626:
551:, vision loss, and shortened lifespan. A loss of vision is common first sign of Batten disease. Loss of vision is typically preceded by cognitive and behavioral changes, seizures, and loss of the ability to walk. It is common for people to establish cardiac arrhythmias and difficulties eating food as the disease progresses. Batten disease diagnosis depends on a conflation of many criteria: clinical signs and symptoms, evaluations of the eye, electroencephalograms (EEG), and brain magnetic resonance imaging (MRI) results. The diagnosis provided by these results are corroborated by genetic and biochemical testing. No effective treatments were available to prevent the disease from being widespread before the past few years. In recent years, more models have been created to expedite the research process for methods to treat Batten disease.
503:) is thought to be the most common known cause of sporadic ALS. Early diagnosis of ALS is harder than with other neurodegenerative diseases as there are no highly effective means of determining its early onset. Currently, there is research being done regarding the diagnosis of ALS through upper motor neuron tests. The Penn Upper Motor Neuron Score (PUMNS) consists of 28 criteria with a score range of 0–32. A higher score indicates a higher level of burden present on the upper motor neurons. The PUMNS has proven quite effective in determining the burden that exists on upper motor neurons in affected patients.
219:. It is the most common neurodegenerative disease. Even with billions of dollars being used to find a treatment for Alzheimer's disease, no effective treatments have been found. Within clinical trials stable and effective AD therapeutic strategies have a 99.5% failure rate. Reasons for this failure rate include inappropriate drug doses, invalid target and participant selection, and inadequate knowledge of pathophysiology of AD. Currently, diagnoses of Alzheimer's is subpar, and better methods need to be utilized for various aspects of clinical diagnoses. Alzheimer's has a 20% misdiagnosis rate.
50:
454:
experience early relapsing and remitting episodes of neuronal deterioration following a period of recovery. Some of these individuals may transition to a more linear progression of the disease, while about 15% of others begin with a progressive course on the onset of multiple sclerosis. The inflammatory response contributes to the loss of the grey matter, and as a result current literature devotes itself to combatting the auto-inflammatory aspect of the disease. While there are several proposed causal links between EBV and the
135:. Neurodegeneration can be found in the brain at many different levels of neuronal circuitry, ranging from molecular to systemic.Because there is no known way to reverse the progressive degeneration of neurons, these diseases are considered to be incurable; however research has shown that the two major contributing factors to neurodegeneration are oxidative stress and inflammation. Biomedical research has revealed many similarities between these diseases at the
6885:
171:
437:, caused by an autoimmune attack resulting in the progressive loss of myelin sheath on neuronal axons. The resultant decrease in the speed of signal transduction leads to a loss of functionality that includes both cognitive and motor impairment depending on the location of the lesion. The progression of MS occurs due to episodes of increasing inflammation, which is proposed to be due to the release of antigens such as
6897:
314:, may play a role in this disease mechanism. Impaired axonal transport of alpha-synuclein may also lead to its accumulation in Lewy bodies. Experiments have revealed reduced transport rates of both wild-type and two familial Parkinson's disease-associated mutant alpha-synucleins through axons of cultured neurons. Membrane damage by alpha-synuclein could be another Parkinson's disease mechanism.
6443:
329:(MAPT) can also cause hereditary PD or increase PD risk. While PD is the second most common neurodegenerative disorder, problems with diagnoses still persist. Problems with the sense of smell is a widespread symptom of Parkinson's disease (PD), however, some neurologists question its efficacy. This assessment method is a source of controversy among medical professionals. The
543:(NCLs) – each caused by a specific gene mutation, of which there are thirteen. Since Batten disease is quite rare, its worldwide prevalence is about 1 in every 100,000 live births. In North America, NCL3 disease (juvenile NCL) typically manifests between the ages of 4 and 7. Batten disease is characterized by motor impairment,
1441:
by
Medivation, Inc. In 2009 this drug was in phase III clinical trials for use in Alzheimer's disease, and also phase II clinical trials for use in Huntington's disease. In March 2010, the results of a clinical trial phase III were released; the investigational Alzheimer's disease drug Dimebon failed
886:
along with enzymes is key for the degradation of many proteins that cause proteopathies including polyQ expansions and alpha-synucleins. Research indicates proteasome enzymes may not be able to correctly cleave these irregular proteins, which could possibly result in a more toxic species. This is the
1470:
A current therapeutic target for the treatment of
Alzheimer's disease is the protease β-secretase, which is involved in the amyloidogenic processing pathway that leads to the pathological accumulation of proteins in the brain. When the gene that encodes for amyloid precursor protein (APP) is spliced
683:
Polyglutamine repeats typically cause dominant pathogenesis. Extra glutamine residues can acquire toxic properties through a variety of ways, including irregular protein folding and degradation pathways, altered subcellular localization, and abnormal interactions with other cellular proteins. PolyQ
1458:
Protein degradation offers therapeutic options both in preventing the synthesis and degradation of irregular proteins. There is also interest in upregulating autophagy to help clear protein aggregates implicated in neurodegeneration. Both of these options involve very complex pathways that we are
918:
is involved with nutrient recycling of macromolecules under conditions of starvation, certain apoptotic pathways, and if absent, leads to the formation of ubiquinated inclusions. Experiments in mice with neuronally confined macroautophagy-gene knockouts develop intraneuronal aggregates leading to
616:
accumulation provides the underlying causative link between aging and neurodegenerative disease. About 20–40% of healthy people between 60 and 78 years old experience discernable decrements in cognitive performance in several domains including working, spatial, and episodic memory, and processing
1436:
of disease to test potential therapeutic agents. Model organisms provide an inexpensive and relatively quick means to perform two main functions: target identification and target validation. Together, these help show the value of any specific therapeutic strategies and drugs when attempting to
1045:
is particularly harmful because DNA is the blueprint for protein production and unlike other molecules it cannot simply be replaced by re-synthesis. The vulnerability of post-mitotic neurons to DNA damage (such as oxidative lesions or certain types of DNA strand breaks), coupled with a gradual
453:
to cross the blood-brain barrier and attack myelin on neuronal axons leading to inflammation. Further release of antigens drives subsequent degeneration causing increased inflammation. Multiple sclerosis presents itself as a spectrum based on the degree of inflammation, a majority of patients
1136:
in any form, mediated by an intracellular program. This process can be activated in neurodegenerative diseases including
Parkinson's disease, amytrophic lateral sclerosis, Alzheimer's disease and Huntington's disease. PCD observed in neurodegenerative diseases may be directly pathogenic;
393:. Huntington's disease presents itself later in life even though the proteins that cause the disease works towards manifestation from their early stages in the humans affected by the proteins. Along with being a neurodegenerative disorder, HD has links to problems with neurodevelopment.
937:
Damage to the membranes of organelles by monomeric or oligomeric proteins could also contribute to these diseases. Alpha-synuclein can damage membranes by inducing membrane curvature, and cause extensive tubulation and vesiculation when incubated with artificial phospholipid vesicles.
611:
both contribute to aging. Many of these diseases are late-onset, meaning there is some factor that changes as a person ages for each disease. One constant factor is that in each disease, neurons gradually lose function as the disease progresses with age. It has been proposed that
283:, rigidity, resting tremor and posture instability. The crude prevalence rate of PD has been reported to range from 15 per 100,000 to 12,500 per 100,000, and the incidence of PD from 15 per 100,000 to 328 per 100,000, with the disease being less common in Asian countries.
1442:
in the pivotal CONNECTION trial of patients with mild-to-moderate disease. With CONCERT, the remaining Pfizer and
Medivation Phase III trial for Dimebon (latrepirdine) in Alzheimer's disease failed in 2012, effectively ending the development in this indication.
487:(LMNs). Although initial symptoms may vary, most patients develop skeletal muscle weakness that progresses to involve the entire body. The precise etiology of ALS remains unknown. In 1993, missense mutations in the gene encoding the antioxidant enzyme
1221:
PCD can also occur via non-apoptotic processes, also known as Type III or cytoplasmic cell death. For example, type III PCD might be caused by trophotoxicity, or hyperactivation of trophic factor receptors. Cytotoxins that induce PCD can cause
1213:, which fuses with a lysosome to destroy the contents of the autophagosome. Because many neurodegenerative diseases show unusual protein aggregates, it is hypothesized that defects in autophagy could be a common mechanism of neurodegeneration.
1445:
In another experiment using a rat model of
Alzheimer's disease, it was demonstrated that systemic administration of hypothalamic proline-rich peptide (PRP)-1 offers neuroprotective effects and can prevent neurodegeneration in hippocampus
1096:
have been observed in many different neurodegenerative diseases. This suggests that defective axons are not only present in diseased neurons, but also that they may cause certain pathological insult due to accumulation of organelles.
1226:
at low concentrations, or aponecrosis (combination of apoptosis and necrosis) at higher concentrations. It is still unclear exactly what combination of apoptosis, non-apoptosis, and necrosis causes different kinds of aponecrosis.
1471:
by α-secretase rather than β-secretase, the toxic protein β amyloid is not produced. Targeted inhibition of β-secretase can potentially prevent the neuronal death that is responsible for the symptoms of
Alzheimer's disease.
5027:
Galoyan AA, Sarkissian JS, Chavushyan VA, Meliksetyan IB, Avagyan ZE, Poghosyan MV, et al. (September 2008). "Neuroprotection by hypothalamic peptide proline-rich peptide-1 in Abeta25-35 model of
Alzheimer's disease".
1466:
is to enhance aspects of the immune system. Both active and passive vaccinations have been proposed for
Alzheimer's disease and other conditions; however, more research must be done to prove safety and efficacy in humans.
2748:
Hill-Burns, Erin M.; Debelius, Justine W.; Morton, James T.; Wissemann, William T.; Lewis, Matthew R.; Wallen, Zachary D.; Peddada, Shyamal D.; Factor, Stewart A.; Molho, Eric; Zabetian, Cyrus P.; Knight, Rob (May 2017).
1148:
is a form of programmed cell death in multicellular organisms. It is one of the main types of programmed cell death (PCD) and involves a series of biochemical events leading to a characteristic cell morphology and death.
925:
defects may also lead to neurodegeneration. Research has shown that mutant proteins bind to the CMA-pathway receptors on lysosomal membrane and in doing so block their own degradation as well as the degradation of other
5289:
4390:
Nabais, Marta F.; Laws, Simon M.; Lin, Tian; Vallerga, Costanza L.; Armstrong, Nicola J.; Blair, Ian P.; Kwok, John B.; Mather, Karen A.; Mellick, George D.; Sachdev, Perminder S.; Wallace, Leanne (2021-03-26).
4003:
Levine, Kristin S.; Leonard, Hampton L.; Blauwendraat, Cornelis; Iwaki, Hirotaka; Johnson, Nicholas; Bandres-Ciga, Sara; Ferrucci, Luigi; Faghri, Faraz; Singleton, Andrew B.; Nalls, Mike A. (19 January 2023).
389:, which initiates and modulates motion. The weaker signals from subthalamic nuclei thus cause reduced initiation and modulation of movement, resulting in the characteristic movements of the disorder, notably
940:
The tubes formed from these lipid vesicles consist of both micellar as well as bilayer tubes. Extensive induction of membrane curvature is deleterious to the cell and would eventually lead to cell death.
2976:
Barnat, Monia; Capizzi, Mariacristina; Aparicio, Esther; Boluda, Susana; Wennagel, Doris; Kacher, Radhia; Kassem, Rayane; Lenoir, Sophie; Agasse, Fabienne; Braz, Barbara Y.; Liu, Jeh-Ping (2020-08-14).
1050:, could lead to accumulation of DNA damage with age and contribute to brain aging and neurodegeneration. DNA single-strand breaks are common and are associated with the neurodegenerative disease ataxia-
668:, most of which are located in completely unrelated genes. In many of the different diseases, the mutated gene has a common feature: a repeat of the CAG nucleotide triplet. CAG codes for the amino acid
890:
Decreased proteasome activity is consistent with models in which intracellular protein aggregates form. It is still unknown whether or not these aggregates are a cause or a result of neurodegeneration.
972:) is a central feature of all neurodegenerative disorders. In addition to the generation of ROS, mitochondria are also involved with life-sustaining functions including calcium homeostasis, PCD,
850:
Parkinson's disease and
Huntington's disease are both late-onset and associated with the accumulation of intracellular toxic proteins. Diseases caused by the aggregation of proteins are known as
3798:
952:
The most common form of cell death in neurodegeneration is through the intrinsic mitochondrial apoptotic pathway. This pathway controls the activation of caspase-9 by regulating the release of
987:
There is strong evidence that mitochondrial dysfunction and oxidative stress play a causal role in neurodegenerative disease pathogenesis, including in four of the more well known diseases
2177:
Svob Strac, Dubravka; Konjevod, Marcela; Sagud, Marina; Nikolac
Perkovic, Matea; Nedic Erjavec, Gordana; Vuic, Barbara; Simic, Goran; Vukic, Vana; Mimica, Ninoslav; Pivac, Nela (2021).
4584:"Chronic oxidative damage together with genome repair deficiency in the neurons is a double whammy for neurodegeneration: Is damage response signaling a potential therapeutic target?"
964:(ROS) are normal byproducts of mitochondrial respiratory chain activity. ROS concentration is mediated by mitochondrial antioxidants such as manganese superoxide dismutase (SOD2) and
5798:
5163:
Esch FS, Keim PS, Beattie EC, Blacher RW, Culwell AR, Oltersdorf T, et al. (June 1990). "Cleavage of amyloid beta peptide during constitutive processing of its precursor".
518:. The specific mechanism of toxicity still needs to be investigated, but the findings are significant because they implicate cells other than neuron cells in neurodegeneration.
6184:
6179:
775:. Alpha-synuclein is the primary structural component of Lewy body fibrils. In addition, an alpha-synuclein fragment, known as the non-Abeta component (NAC), is found in
6196:
6174:
4946:
Caccamo D, Currò M, Condello S, Ferlazzo N, Ientile R (February 2010). "Critical role of transglutaminase and other stress proteins during neurodegenerative processes".
162:
The consequences of neurodegeneration can vary widely depending on the specific region affected, ranging from issues related to movement to the development of dementia.
3320:
Quinn C, Edmundson C, Dahodwala N, Elman L (April 2020). "Reliable and efficient scale to assess upper motor neuron disease burden in amyotrophic lateral sclerosis".
2321:
Turner PR, O'Connor K, Tate WP, Abraham WC (May 2003). "Roles of amyloid precursor protein and its fragments in regulating neural activity, plasticity and memory".
1197:. Initiator caspases cleave inactive forms of effector caspases. This activates the effectors that in turn cleave other proteins resulting in apoptotic initiation.
902:(PCD), this becomes the favorable route when a protein is aggregate-prone meaning it is a poor proteasome substrate. This can be split into two forms of autophagy:
5347:
5120:
Pastorino L, Ikin AF, Lamprianou S, Vacaresse N, Revelli JP, Platt K, et al. (April 2004). "BACE (beta-secretase) modulates the processing of APLP2 in vivo".
3086:
684:
studies often use a variety of animal models because there is such a clearly defined trigger – repeat expansion. Extensive research has been done using the
458:
allele to the onset of MS – they may contribute to the degree of autoimmune attack and the resultant inflammation – they do not determine the onset of MS.
5257:
1275:
of these proteins and peptides make them clump together. The resulting structures are turned extremely resistant to chemical and mechanical disruption.
572:
disease that is characterized by rapidly progressive dementia. Misfolded proteins called prions aggregate in brain tissue leading to nerve cell death.
2399:
Tiraboschi P, Hansen LA, Thal LJ, Corey-Bloom J (June 2004). "The importance of neuritic plaques and tangles to the development and evolution of AD".
5543:
3746:
1635:
5538:
1505:
2751:"Parkinson's disease and Parkinson's disease medications have distinct signatures of the gut microbiome: PD, Medications, and Gut Microbiome"
6805:
5340:
625:
246:
that penetrates through the neuron's membrane. APP appears to play roles in normal neuron growth, survival and post-injury repair. APP is
17:
3772:
3570:
2829:
Estrada Sánchez AM, Mejía-Toiber J, Massieu L (April 2008). "Excitotoxic neuronal death and the pathogenesis of Huntington's disease".
1539:
807:
6166:
2364:
Hooper NM (April 2005). "Roles of proteolysis and lipid rafts in the processing of the amyloid precursor protein and prion protein".
1365:
It has been proved that in these neurodegenerative diseases (Alzheimer's disease, Parkinson's disease, and Huntington's disease) the
1063:
1059:
868:
endoplasmic reticulum (ER), (as seen with neuroserpin mutations that cause familial encephalopathy with neuroserpin inclusion bodies)
659:
262:. One of these fragments gives rise to fibrils of amyloid beta which can self-assemble into the dense extracellular amyloid plaques.
3606:
Johnson, Tyler B.; Cain, Jacob T.; White, Katherine A.; Ramirez-Montealegre, Denia; Pearce, David A.; Weimer, Jill M. (March 2019).
2803:
535:
is a rare and fatal recessive neurodegenerative disorder that begins in childhood. Batten disease is the common name for a group of
333:
might play a role in the diagnosis of PD, and research suggests various ways that could revolutionize the future of PD treatment.
6156:
5333:
1662:"Whole-Brain Dynamics Disruptions in the Progression of Alzheimer's Disease: Understanding the Influence of Amyloid-Beta and Tau"
833:
4097:
1707:"The Emerging Scenario of the Gut-Brain Axis: The Therapeutic Actions of the New Actor Kefir against Neurodegenerative Diseases"
404:
in neurons, and may be directly toxic. Additionally, they may damage molecular motors and microtubules to interfere with normal
6277:
6136:
5809:
1586:"A Review of the Common Neurodegenerative Disorders: Current Therapeutic Approaches and the Potential Role of Nanotherapeutics"
1055:
6161:
5793:
3886:
3123:
2813:
2153:
2065:
2033:
573:
174:
Comparison of brain tissue between healthy individual and Alzheimer's disease patient, demonstrating extent of neuronal death
6938:
5999:
5987:
5449:
719:
The presence of epigenetic modifications for certain genes has been demonstrated in this type of pathology. An example is
703:
Nine inherited neurodegenerative diseases are caused by the expansion of the CAG trinucleotide and polyQ tract, including
641:) viral exposures can significantly elevate risks of neurodegenerative disease, including up to 15 years after infection.
438:
604:
6218:
6151:
5706:
1156:
Occur when factors outside the cell activate cell surface death receptors (e.g., Fas) that result in the activation of
449:, causing an autoimmune response. This sets off a cascade of signaling molecules that result in T cells, B cells, and
6785:
5921:
5724:
1705:
Pereira, TMC; Côco, LZ; Ton, AMM; Meyrelles, SS; Campos-Toimil, M; Campagnaro, BP; Vasquez, EC (20 November 2021).
1420:
The process of neurodegeneration is not well understood, so the diseases that stem from it have, as yet, no cures.
1034:
613:
577:
943:
Apart from tubular structures, alpha-synuclein can also form lipoprotein nanoparticles similar to apolipoproteins.
6928:
6040:
4393:"Meta-analysis of genome-wide DNA methylation identifies shared associations across neurodegenerative disorders"
3826:
Lin MT, Beal MF (October 2006). "Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases".
2552:
De Vos KJ, Grierson AJ, Ackerley S, Miller CC (2008). "Role of axonal transport in neurodegenerative diseases".
2020:
6923:
5555:
5531:
5361:
2233:"[P2–430]: Accuracy of Clinical Diagnosis of Alzheimer's Disease in Alzheimer's Disease Centers (ADCS)"
1899:
Rubinsztein DC (October 2006). "The roles of intracellular protein-degradation pathways in neurodegeneration".
540:
2699:"Invalid Self-Assessment of Olfactory Functioning in Parkinson's Disease Patients May Mislead the Neurologist"
400:
expansion in the huntingtin gene, resulting in the mutant huntingtin. Aggregates of mutant huntingtin form as
6901:
6626:
6234:
6119:
6035:
5977:
5747:
5526:
1000:
862:
677:
655:
565:
472:
467:
104:
4341:"Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1"
3258:"Dissociated leg muscle atrophy in amyotrophic lateral sclerosis/motor neuron disease: the 'split-leg' sign"
6780:
6601:
6203:
5577:
2916:
310:
within affected neurons. It is thought that defects in protein transport machinery and regulation, such as
6820:
5713:
1209:
in which a cell actively consumes damaged organelles or misfolded proteins by encapsulating them into an
6486:
5258:"Colorado doctor prescribes ping pong treatment for neurodegenerative disorders: 'Doing something good'"
3111:
3110:
Kaufman, David Myland; Milstein, Mark J. (2013-01-01), Kaufman, David Myland; Milstein, Mark J. (eds.),
1636:"Brain Basics: The Life and Death of a Neuron | National Institute of Neurological Disorders and Stroke"
6347:
6270:
6191:
5741:
5413:
1475:
536:
499:
protein aggregates have been implicated in some cases of the disease, and a mutation in chromosome 9 (
6790:
6659:
6141:
6109:
6083:
5910:
3087:"Multiple Sclerosis: Hope Through Research | National Institute of Neurological Disorders and Stroke"
980:, lipid concentration of the mitochondrial membranes, and the mitochondrial permeability transition.
768:
560:
239:
4530:"Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications"
3218:
1285:
Each of these neurodegenerative diseases have one (or several) specific main protein or peptide. In
6754:
6729:
6654:
6561:
6466:
6362:
6208:
5982:
5396:
5308:
4837:
Vila M, Przedborski S (May 2003). "Targeting programmed cell death in neurodegenerative diseases".
2594:"Membrane curvature induction and tubulation are common features of synucleins and apolipoproteins"
1062:. Defective DNA repair has been linked to neurodegenerative disorders such as Alzheimer's disease,
984:
leading to neurodegeneration is likely, at least on some level, to involve all of these functions.
634:
4290:"Unstable nucleotide repeat minireview series: a molecular biography of unstable repeat disorders"
3365:"Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons"
6591:
6302:
6297:
6213:
6146:
5902:
5720:
5572:
5562:
5406:
2645:"Variants in GBA, SNCA, and MAPT influence Parkinson disease risk, age at onset, and progression"
1030:
1014:
961:
957:
799:
772:
704:
347:
342:
124:
120:
5325:
3747:"Creutzfeldt-Jakob Disease Fact Sheet | National Institute of Neurological Disorders and Stroke"
1178:
are other organelles that have damage sensors, which can lead the cells down apoptotic pathways.
6586:
6576:
6367:
6322:
6078:
6070:
6025:
5701:
5673:
5521:
3662:
Masten MC, Williams JD, Vermilion J, Adams HR, Vierhile A, Collins A, et al. (June 2020).
3414:"Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model"
3213:
1510:
1286:
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965:
780:
708:
434:
276:
271:
227:
184:
179:
116:
112:
62:
31:
2499:
Elbaz A, Carcaillon L, Kab S, Moisan F (January 2016). "Epidemiology of Parkinson's disease".
875:
There are two main avenues eukaryotic cells use to remove troublesome proteins or organelles:
6889:
6649:
6644:
6451:
6342:
6263:
6093:
5967:
5785:
5763:
5678:
5601:
5511:
5498:
5423:
3363:
Nagai M, Re DB, Nagata T, Chalazonitis A, Jessell TM, Wichterle H, Przedborski S (May 2007).
2798:
Purves D, Augustine GA, Fitzpatrick D, Hall W, LaMantia AS, McNamara JO, Williams SM (2001).
1483:
1341:
1320:
1129:
1075:
1067:
981:
973:
899:
430:
243:
412:. Huntington's disease currently has no effective treatments that would modify the disease.
6870:
6621:
6461:
6432:
6327:
6030:
5847:
5736:
5172:
5088:
4582:
Wang H, Dharmalingam P, Vasquez V, Mitra J, Boldogh I, Rao KS, et al. (January 2017).
4506:
4188:
4065:
3835:
3269:
2990:
2565:
1976:
1908:
977:
442:
3799:"Variant Creutzfeldt-Jakob Disease (vCJD) and Factor VIII (pdFVIII) Questions and Answers"
8:
6933:
6764:
6664:
6541:
6511:
6496:
5959:
5606:
5461:
5401:
2660:
2643:
Davis AA, Andruska KM, Benitez BA, Racette BA, Perlmutter JS, Cruchaga C (January 2016).
1409:
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792:
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673:
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397:
382:
362:
49:
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2994:
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2468:
2443:
1980:
1912:
1682:
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or endoplasmic reticulum malfunctions, each leading to the activation of caspase-9. The
479:
disease, is a rare neurodegenerative disorder characterized by the gradual loss of both
238:(also written as A-beta or Aβ). Amyloid beta is a fragment from a larger protein called
150:
Within neurodegenerative diseases, it is estimated that 55 million people worldwide had
147:
advances against one neurodegenerative disease might ameliorate other diseases as well.
6840:
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5852:
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4714:
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2725:
2698:
2669:
2644:
2620:
2593:
2592:
Varkey J, Isas JM, Mizuno N, Jensen MB, Bhatia VK, Jao CC, et al. (October 2010).
2481:
2424:
2412:
2346:
2298:
2273:
2254:
2205:
2178:
1997:
1964:
1932:
1833:
1806:
1782:
1757:
1733:
1706:
1612:
1585:
1561:
1534:
1106:
1051:
576:(vCJD) is the infectious form that comes from the meat of a cow that was infected with
484:
480:
426:
421:
351:
322:
108:
2866:"Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease"
2697:
Schmidt, Nele; Paschen, Laura; Witt, Karsten (2020-11-16). Mirabella, Giovanni (ed.).
2334:
1856:
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4093:
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4052:
4037:
3982:
3933:
3882:
3851:
3732:
3693:
3664:"The CLN3 Disease Staging System: A new tool for clinical research in Batten disease"
3637:
3571:"Batten Disease Fact Sheet | National Institute of Neurological Disorders and Stroke"
3541:
3500:
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3443:
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3065:
3016:
2955:
2895:
2846:
2809:
2780:
2730:
2674:
2625:
2569:
2516:
2473:
2416:
2381:
2338:
2303:
2210:
2159:
2149:
2124:
2097:
2061:
2029:
2002:
1924:
1838:
1787:
1738:
1687:
1661:
1617:
1566:
1345:
1249:
1071:
279:(PD) is the second most common neurodegenerative disorder. It typically manifests as
70:
5149:
5057:
4975:
4866:
4705:
2485:
2428:
2350:
2258:
1356:
to each other and potentially to any other transglutaminase substrate in the brain.
763:
can aggregate to form insoluble fibrils in pathological conditions characterized by
723:
gene, which progressively increases its expression with age and has been related to
6800:
6684:
6611:
6526:
6407:
6357:
6015:
5886:
5881:
5666:
5661:
5229:
5219:
5180:
5129:
5092:
5084:
5037:
5009:
4955:
4901:
4893:
4846:
4809:
4799:
4758:
4750:
4709:
4701:
4655:
4647:
4603:
4595:
4551:
4541:
4502:
4465:
4455:
4414:
4404:
4362:
4352:
4311:
4301:
4260:
4250:
4196:
4144:
4134:
4073:
4027:
4017:
3972:
3964:
3923:
3915:
3863:
3843:
3720:
3683:
3675:
3627:
3619:
3608:"Therapeutic landscape for Batten disease: current treatments and future prospects"
3553:
3531:
3490:
3474:
3433:
3425:
3384:
3376:
3329:
3285:
3277:
3223:
3172:
3167:
3162:
3055:
3047:
3006:
2998:
2945:
2937:
2885:
2877:
2838:
2770:
2762:
2720:
2710:
2664:
2656:
2615:
2605:
2561:
2508:
2463:
2455:
2408:
2373:
2330:
2293:
2289:
2285:
2244:
2200:
2190:
2089:
2060:. Focus on structural biology. Dordrecht, Netherlands, New York, London: Springer.
1992:
1984:
1936:
1916:
1828:
1818:
1777:
1769:
1728:
1718:
1677:
1669:
1607:
1597:
1556:
1548:
1391:
1370:
1360:
1317:
1235:
1098:
1087:
1010:
969:
753:. Protein toxicity is one of the key mechanisms of many neurodegenrative diseases.
665:
608:
405:
401:
291:
2534:
1584:
Lamptey RN, Chaulagain B, Trivedi R, Gothwal A, Layek B, Singh J (February 2022).
317:
The main known risk factor is age. Mutations in genes such as α-synuclein (SNCA),
61:
on left contrasted with structural changes shown in brain on right of person with
6860:
6855:
6850:
6845:
6739:
6679:
6616:
6531:
6491:
6476:
6427:
6417:
6372:
5937:
5628:
5313:
5000:
Sweetlove M (2012). "Phase III CONCERT Trial of Latrepirdine. Negative results".
4988:
4804:
4651:
4139:
4022:
3679:
2842:
2512:
2272:
Priller C, Bauer T, Mitteregger G, Krebs B, Kretzschmar HA, Herms J (July 2006).
1429:
1405:
1387:
1381:
1366:
1333:
1306:
1302:
1265:
1261:
1257:
1175:
1093:
996:
776:
758:
750:
510:
evidence that the primary cellular sites where SOD1 mutations act are located on
386:
355:
299:
255:
223:
216:
196:
5224:
3919:
3478:
2249:
2232:
1552:
6825:
6795:
6724:
6699:
6689:
6536:
6516:
6506:
6392:
6312:
5994:
5943:
5771:
5480:
5357:
5041:
4409:
4077:
3281:
3051:
2799:
1396:
abnormal structures that are characteristic of these neurodegenerative diseases
1353:
1298:
1253:
1137:
alternatively, PCD may occur in response to other injury or disease processes.
1133:
992:
988:
942:
939:
904:
829:
736:
685:
532:
527:
330:
259:
212:
132:
5133:
4959:
4599:
4460:
3902:
Maynard S, Fang EF, Scheibye-Knudsen M, Croteau DL, Bohr VA (September 2015).
3623:
3202:"Remyelination protects axons from demyelination-associated axon degeneration"
2459:
2274:"Synapse formation and function is modulated by the amyloid precursor protein"
1823:
1673:
1117:. When axonal transport is severely disrupted a degenerative pathway known as
6917:
6749:
6744:
6709:
6606:
6596:
6556:
6402:
6337:
6332:
6020:
5972:
5842:
5611:
5567:
5418:
4882:"Pharmacological manipulation of cell death: clinical applications in sight?"
4493:
DiMauro S, Schon EA (2008). "Mitochondrial disorders in the nervous system".
3486:
3036:"Huntington's disease: underlying molecular mechanisms and emerging concepts"
2163:
2101:
1535:"Clinical Neurology and Epidemiology of the Major Neurodegenerative Diseases"
1500:
1463:
1349:
1272:
1210:
851:
724:
515:
378:
208:
204:
140:
5302:
5298:
5184:
4006:"Virus exposure and neurodegenerative disease risk across national biobanks"
3968:
3724:
3002:
2610:
2093:
1660:
Patow, Gustavo; Escrichs, Anira; Ritter, Petra; Deco, Gustavo (2024-03-31).
1412:
has been detected in the autopsy of brains of patients with these diseases.
1278:
Most relevant human neurodegenerative diseases share the property of having
6835:
6830:
6714:
6546:
6471:
6422:
6412:
6387:
6382:
6352:
6286:
5516:
5386:
5369:
5243:
5141:
5106:
5049:
4967:
4915:
4858:
4823:
4772:
4723:
4669:
4617:
4565:
4546:
4514:
4479:
4428:
4376:
4357:
4325:
4306:
4274:
4255:
4210:
4158:
4085:
4041:
3986:
3937:
3855:
3697:
3641:
3545:
3504:
3447:
3398:
3341:
3299:
3237:
3228:
3201:
3186:
3069:
3020:
2959:
2899:
2850:
2784:
2734:
2715:
2678:
2629:
2573:
2520:
2477:
2420:
2385:
2342:
2307:
2214:
2128:
2006:
1928:
1842:
1791:
1742:
1723:
1691:
1621:
1570:
1479:
1447:
1433:
1325:
1290:
1242:
1206:
1171:
1167:
1114:
1110:
953:
854:, and they are primarily caused by aggregates in the following structures:
814:
374:
366:
287:
280:
235:
154:
in 2019, and that by 2050 this figure will increase to 139 million people.
5192:
4123:"Viruses in neurodegenerative diseases: More than just suspects in crimes"
2179:"Personalizing the Care and Treatment of Alzheimer's Disease: An Overview"
2143:
2081:
491:
were discovered in a subset of patients with familial ALS. More recently,
6704:
6551:
5475:
5436:
5378:
2195:
1602:
1018:
803:
795:
787:
764:
742:
594:
326:
307:
247:
136:
58:
5281:
4788:"Bacterial programmed cell death and multicellular behavior in bacteria"
3847:
1988:
1920:
187:(AD) is a chronic neurodegenerative disease that results in the loss of
6865:
6566:
5586:
5456:
5013:
3536:
3519:
2377:
2176:
1533:
Erkkinen, Michael G.; Kim, Mee-Ohk; Geschwind, Michael D (April 2018).
1337:
1310:
1186:
1047:
638:
496:
476:
450:
100:
79:
5026:
4785:
3333:
2766:
1773:
871:
extracellularly excreted proteins, amyloid-beta in Alzheimer's disease
6810:
6501:
5696:
5647:
5635:
5618:
5550:
4897:
4786:
Engelberg-Kulka H, Amitai S, Kolodkin-Gal I, Hazan R (October 2006).
4053:"Massive health-record review links viral illnesses to brain disease"
3412:
Di Giorgio FP, Carrasco MA, Siao MC, Maniatis T, Eggan K (May 2007).
3256:
Min YG, Choi SJ, Hong YH, Kim SM, Shin JY, Sung JJ (September 2020).
1450:
25–35. This suggests that there could be therapeutic value to PRP-1.
1145:
883:
727:
and increased tau pathology both in vitro and in mouse models of AD.
669:
511:
303:
128:
75:
4850:
4239:"Animal models of polyglutamine diseases and therapeutic approaches"
4201:
4176:
6719:
6171:
Spinal muscular atrophy with lower extremity predominance (SMALED)
5876:
5868:
5834:
5688:
5591:
5261:
3605:
3429:
3380:
2881:
1223:
1182:
1157:
689:
548:
544:
370:
295:
170:
151:
5073:"Active and passive immunotherapy for neurodegenerative disorders"
3901:
2828:
2145:
The Alzheimer conundrum : entanglements of dementia and aging
2115:
Wenk GL (2003). "Neuropathologic changes in Alzheimer's disease".
5355:
2747:
1807:"Oxidative Stress: A Key Modulator in Neurodegenerative Diseases"
1495:
1438:
1239:
1102:
1101:
can be disrupted by a variety of mechanisms including damage to:
1054:. Increased oxidative DNA damage in the brain is associated with
1029:
The brain metabolizes as much as a fifth of consumed oxygen, and
500:
251:
231:
200:
192:
144:
5119:
3116:
Kaufman's Clinical Neurology for Psychiatrists (Seventh Edition)
1252:
proteins and peptides intra- and intermolecularly, by a type of
6255:
5915:
5656:
5623:
5293:
3998:
3996:
3797:
Research, Center for Biologics Evaluation and (12 April 2019).
2398:
1756:
Stephenson, J; Nutma, E; van der Valk, P; Amor, S (June 2018).
1755:
1583:
1373:
1006:
600:
492:
390:
188:
92:
4444:"Mechanisms of protein toxicity in neurodegenerative diseases"
4002:
3411:
2022:
Global status report on the public health response to dementia
819:
the major component of amyloid plaques in Alzheimer's disease.
802:
in Alzheimer's disease; tau fibrils are the main component of
637:
indicates that 45 (with 22 of these being replicated with the
298:. The cause of this selective cell death is unknown. Notably,
2320:
1038:
823:
720:
569:
408:, leading to impaired transport of important cargoes such as
369:. The first brain region to be substantially affected is the
318:
4945:
4581:
3993:
3661:
3319:
2551:
2271:
1013:
due to their strong metabolic activity associated with high
2975:
2642:
1805:
Singh, A; Kukreti, R; Saso, L; Kukreti, S (22 April 2019).
1185:(cysteine-aspartic acid proteases) cleave at very specific
599:
The greatest risk factor for neurodegenerative diseases is
488:
409:
311:
222:
AD pathology is primarily characterized by the presence of
6442:
3953:"Brain metabolism in health, aging, and neurodegeneration"
2058:
Protein folding and misfolding: neurodegenerative diseases
1245:
present in the human body and in the brain in particular.
143:) and induced cell death. These similarities suggest that
3362:
1329:
1294:
1042:
629:
Risks from viral exposures according to one biobank study
4236:
2498:
4527:
2444:"Amyloid fibrils from the viewpoint of protein folding"
1804:
1704:
1659:
1033:
produced by oxidative metabolism are a major source of
676:. Diseases associated with such mutations are known as
306:
complexes and aggregates are observed to accumulate in
199:
and certain subcortical structures, resulting in gross
5162:
4528:
Liu Z, Zhou T, Ziegler AC, Dimitrion P, Zuo L (2017).
3904:"DNA Damage, DNA Repair, Aging, and Neurodegeneration"
2591:
1428:
In the search for effective treatments (as opposed to
354:
neurodegenerative disorder caused by mutations in the
99:. Neuronal damage may also ultimately result in their
4687:
4389:
3151:"Recent advances in understanding multiple sclerosis"
741:
Several neurodegenerative diseases are classified as
234:, typically 39–43 amino acids in length, called
5271:
2979:"Huntington's disease alters human neurodevelopment"
2808:(2nd ed.). Sunderland, MA: Sinauer Associates.
1532:
1437:
ameliorate disease severity. An example is the drug
1280:
abnormal structures made up of proteins and peptides
4237:Marsh JL, Lukacsovich T, Thompson LM (March 2009).
2013:
139:level, including atypical protein assemblies (like
5208:"Treatment strategies targeting amyloid β-protein"
5205:
4051:
3876:
2696:
5206:Schenk D, Basi GS, Pangalos MN (September 2012).
4633:
4121:Leblanc, Pascal; Vorberg, Ina Maja (2022-08-04).
3773:"Creutzfeldt-Jakob disease - Symptoms and causes"
3118:, Philadelphia: W.B. Saunders, pp. 329–349,
2863:
1962:
1453:
514:. Astrocytes then cause the toxic effects on the
6915:
3950:
3460:
3081:
3079:
3033:
2230:
1758:"Inflammation in CNS neurodegenerative diseases"
461:
4836:
4688:Jeppesen DK, Bohr VA, Stevnsner T (July 2011).
4683:
4681:
4679:
4636:"DNA damage and its links to neurodegeneration"
4629:
4627:
4577:
4575:
3881:. San Diego: Academic Press. pp. 121–139.
3199:
3149:Stys, Peter K.; Tsutsui, Shigeki (2019-12-13).
3109:
2692:
2690:
2688:
845:
4736:
4120:
3657:
3655:
3653:
3651:
3255:
2971:
2969:
2226:
2224:
1963:Bredesen DE, Rao RV, Mehlen P (October 2006).
1506:Neurodegeneration with brain iron accumulation
1482:for patients who are suffering from a serious
664:Many neurodegenerative diseases are caused by
6271:
5341:
5070:
4442:Chung, CG; Lee, H; Lee, SB (September 2018).
3870:
3520:"ALS: astrocytes move in as deadly neighbors"
3461:Phatnani, Hemali; Maniatis, Tom (June 2015).
3315:
3313:
3311:
3309:
3076:
2441:
1894:
1892:
1890:
1888:
1886:
1884:
1882:
1880:
1878:
1528:
1526:
1404:Co-localization of transglutaminase mediated
887:primary route cells use to degrade proteins.
6806:Intraoperative neurophysiological monitoring
4879:
4690:"DNA repair deficiency in neurodegeneration"
4676:
4624:
4572:
4521:
4492:
3944:
3895:
3601:
3599:
3597:
3595:
3593:
3591:
3144:
3142:
3140:
2921:
2857:
2822:
2685:
2636:
2587:
2585:
2583:
2533:"Parkinson's Disease Mechanism Discovered,"
2435:
2392:
2357:
2314:
2265:
2108:
1423:
947:
554:
6060:
5212:Cold Spring Harbor Perspectives in Medicine
4993:
4739:"Wallerian degeneration, wld(s), and nmnat"
4441:
4170:
4168:
3908:Cold Spring Harbor Perspectives in Medicine
3711:Hartnett L (2019-09-30). "Batten disease".
3648:
3251:
3249:
3247:
3148:
2966:
2864:Lobsiger CS, Cleveland DW (November 2007).
2221:
2028:. Geneva: World Health Organization. 2021.
1958:
1956:
1954:
1952:
1950:
1948:
1946:
1898:
1189:residues. There are two types of caspases:
840:
65:, the most common neurodegenerative disease
6278:
6264:
5348:
5334:
4941:
4939:
4937:
4935:
4933:
4931:
4929:
4927:
4925:
4634:Madabhushi R, Pan L, Tsai LH (July 2014).
4177:"Neurodegeneration: a question of balance"
3467:Cold Spring Harbor Perspectives in Biology
3306:
2930:"Functional anatomy of movement disorders"
2800:"Circuits within the Basal Ganglia System"
2183:Pharmacogenomics and Personalized Medicine
2055:
1875:
1540:Cold Spring Harbor Perspectives in Biology
1523:
1348:in vitro or in vivo, that is, they can be
1248:The main function of transglutaminases is
1017:levels, high oxygen consumption, and weak
858:cytosol, e.g. Parkinson's and Huntington's
808:behavioral variant frontotemporal dementia
286:PD is primarily characterized by death of
48:
5233:
5223:
5096:
4999:
4905:
4813:
4803:
4779:
4762:
4713:
4659:
4607:
4555:
4545:
4534:Oxidative Medicine and Cellular Longevity
4469:
4459:
4418:
4408:
4366:
4356:
4338:
4315:
4305:
4264:
4254:
4200:
4148:
4138:
4031:
4021:
3976:
3927:
3687:
3631:
3588:
3535:
3494:
3463:"Astrocytes in Neurodegenerative Disease"
3437:
3405:
3388:
3289:
3227:
3217:
3176:
3166:
3137:
3059:
3010:
2949:
2889:
2774:
2724:
2714:
2668:
2619:
2609:
2580:
2467:
2297:
2248:
2204:
2194:
1996:
1832:
1822:
1781:
1749:
1732:
1722:
1681:
1611:
1601:
1560:
1216:
660:Epigenetics of neurodegenerative diseases
4232:
4230:
4228:
4174:
4165:
3796:
3710:
3244:
3200:Irvine, K. A.; Blakemore, W. F. (2008).
2927:
2547:
2545:
2543:
1943:
1124:
624:
169:
5020:
4922:
4486:
3825:
3034:Labbadia J, Morimoto RI (August 2013).
1200:
834:transmissible spongiform encephalopathy
336:
14:
6916:
6137:Distal hereditary motor neuronopathies
5810:Template:Demyelinating diseases of CNS
5089:10.1146/annurev.neuro.31.060407.125529
4830:
4507:10.1146/annurev.neuro.30.051606.094302
4049:
3821:
3819:
3565:
3563:
3517:
3511:
2912:
2797:
2566:10.1146/annurev.neuro.31.061307.090711
2363:
2231:Archer MC, Hall PH, Morgan JC (2017).
1698:
1577:
265:
165:
6259:
5329:
5064:
4886:The Journal of Clinical Investigation
4873:
4435:
4225:
3951:Camandola S, Mattson MP (June 2017).
3356:
2540:
1205:Autophagy is a form of intracellular
1166:Result from mitochondrial release of
1140:
730:
415:
157:
103:. Neurodegenerative diseases include
91:is caused by the progressive loss of
6896:
5988:Idiopathic intracranial hypertension
5377:
5122:Molecular and Cellular Neurosciences
4989:Dimebon Disappoints in Phase 3 Trial
4880:Green DR, Kroemer G (October 2005).
4588:Mechanisms of Ageing and Development
4448:Cellular and Molecular Life Sciences
4100:from the original on 6 February 2023
2661:10.1016/j.neurobiolaging.2015.09.014
2448:Cellular and Molecular Life Sciences
2141:
2114:
2056:Ovádi, Judit; Orosz, Ferenc (2009).
1857:"What is Neurodegenerative Disease?"
1798:
1628:
1394:reaction) have been detected in the
4755:10.1146/annurev-neuro-060909-153248
4345:The Journal of Biological Chemistry
4294:The Journal of Biological Chemistry
4287:
4281:
4243:The Journal of Biological Chemistry
3816:
3560:
2942:10.1046/j.1469-7580.2000.19640519.x
2598:The Journal of Biological Chemistry
1849:
1230:
1081:
493:TAR DNA-binding protein 43 (TDP-43)
439:myelin oligodendrocyte glycoprotein
24:
6219:Infantile progressive bulbar palsy
2442:Ohnishi S, Takano K (March 2004).
2413:10.1212/01.WNL.0000129697.01779.0A
2117:The Journal of Clinical Psychiatry
1965:"Cell death in the nervous system"
932:
910:chaperone-mediated autophagy (CMA)
373:, followed by degeneration of the
25:
6950:
6786:Development of the nervous system
5922:Template:Cerebrovascular diseases
5725:Frontotemporal lobar degeneration
3877:Bernstein C, Bernstein H (1991).
3112:"Chapter 15 - Multiple Sclerosis"
958:mitochondrial intermembrane space
700:), mice, and non-human primates.
574:Variant Creutzfeldt–Jakob disease
521:
361:. HD is characterized by loss of
6895:
6884:
6883:
6441:
6285:
5942:For more detailed coverage, see
5920:For more detailed coverage, see
5891:For more detailed coverage, see
5857:For more detailed coverage, see
5808:For more detailed coverage, see
4339:Zoghbi HY, Orr HT (March 2009).
2366:Biochemical Society Transactions
2086:Cell Biochemistry and Biophysics
745:as they are associated with the
578:bovine spongiform encephalopathy
5250:
5199:
5156:
5113:
4982:
4737:Coleman MP, Freeman MR (2010).
4730:
4706:10.1016/j.pneurobio.2011.04.013
4383:
4332:
4114:
4050:Kozlov, Max (23 January 2023).
3790:
3765:
3739:
3704:
3454:
3193:
3103:
3027:
2906:
2791:
2741:
2527:
2492:
2170:
2135:
2074:
2049:
1009:are particularly vulnerable to
767:, such as Parkinson's disease,
672:. A repeat of CAG results in a
583:
580:, also called mad cow disease.
429:(MS) is a chronic debilitating
230:. Plaques are made up of small
5071:Brody DL, Holtzman DM (2008).
4048:News article about the study:
3753:. National Institute of Health
3168:10.12688/f1000research.20906.1
3040:Trends in Biochemical Sciences
2290:10.1523/JNEUROSCI.1450-06.2006
2148:. Princeton University Press.
1653:
1474:Dr. Antonio Barbera, a former
1459:only beginning to understand.
1454:Other avenues of investigation
714:
678:trinucleotide repeat disorders
541:neuronal ceroid lipofuscinoses
506:Independent research provided
27:Central nervous system disease
13:
1:
6627:Social cognitive neuroscience
6235:Amyotrophic lateral sclerosis
6120:Hereditary spastic paraplegia
5978:Normal pressure hydrocephalus
5077:Annual Review of Neuroscience
4495:Annual Review of Neuroscience
2554:Annual Review of Neuroscience
2335:10.1016/S0301-0082(03)00089-3
1516:
1415:
1164:Intrinsic apoptotic pathways:
1154:Extrinsic apoptotic pathways:
1064:amyotrophic lateral sclerosis
1046:decline in the activities of
1024:
1001:amyotrophic lateral sclerosis
863:Spinocerebellar ataxia type 1
656:Trinucleotide repeat disorder
644:
620:
489:superoxide dismutase 1 (SOD1)
473:Amyotrophic lateral sclerosis
468:Amyotrophic lateral sclerosis
462:Amyotrophic lateral sclerosis
105:amyotrophic lateral sclerosis
30:For the medical journal, see
6602:Molecular cellular cognition
6204:Progressive muscular atrophy
4839:Nature Reviews. Neuroscience
4805:10.1371/journal.pgen.0020135
4652:10.1016/j.neuron.2014.06.034
4140:10.1371/journal.ppat.1010670
4023:10.1016/j.neuron.2022.12.029
3713:Learning Disability Practice
3680:10.1212/WNL.0000000000009454
2843:10.1016/j.arcmed.2007.11.011
2831:Archives of Medical Research
2513:10.1016/j.neurol.2015.09.012
923:chaperone-mediated autophagy
896:autophagy–lysosome pathways:
846:Protein degradation pathways
475:(ALS), commonly referred to
385:send control signals to the
319:leucine-rich repeat kinase 2
7:
6939:Neurodegenerative disorders
6821:Neurodevelopmental disorder
6796:Neural network (biological)
6791:Neural network (artificial)
5714:Primary progressive aphasia
5225:10.1101/cshperspect.a006387
3920:10.1101/cshperspect.a025130
3479:10.1101/cshperspect.a020628
2936:. 196 ( Pt 4) (4): 519–25.
2917:Box A. Huntington's Disease
2278:The Journal of Neuroscience
2250:10.1016/j.jalz.2017.06.1086
2142:Lock MM (27 October 2013).
1553:10.1101/cshperspect.a033118
1489:
1119:Wallerian-like degeneration
674:polyglutamine (polyQ) tract
649:
605:Mitochondrial DNA mutations
537:lysosomal storage disorders
18:Neurodegenerative disorders
10:
6955:
6348:Computational neuroscience
6036:Hashimoto's encephalopathy
5742:Posterior cortical atrophy
5563:Striatonigral degeneration
5414:Cavernous sinus thrombosis
5042:10.1016/j.jalz.2007.10.019
5030:Alzheimer's & Dementia
4410:10.1186/s13059-021-02275-5
4175:Thompson LM (April 2008).
4078:10.1038/d41586-023-00181-3
3879:Aging, Sex, and DNA Repair
3282:10.1038/s41598-020-72887-7
3052:10.1016/j.tibs.2013.05.003
2237:Alzheimer's & Dementia
1476:obstetrics and gynaecology
1085:
1074:, Parkinson's disease and
968:. Over production of ROS (
734:
653:
592:
558:
525:
465:
419:
340:
269:
250:into smaller fragments by
177:
95:, in the process known as
29:
6879:
6816:Neurodegenerative disease
6773:
6660:Evolutionary neuroscience
6635:
6575:
6450:
6439:
6311:
6293:
6142:Spinal muscular atrophies
6110:Primary lateral sclerosis
6092:
6069:
6053:
6008:
5968:Intracranial hypertension
5958:
5930:
5901:
5867:
5829:
5818:
5784:
5762:
5748:Creutzfeldt–Jakob disease
5687:
5497:
5488:
5474:
5434:
5368:
5275:
5134:10.1016/j.mcn.2003.12.013
4960:10.1007/s00726-009-0428-3
4600:10.1016/j.mad.2016.09.005
4461:10.1007/s00018-018-2854-4
3624:10.1038/s41582-019-0138-8
2460:10.1007/s00018-003-3264-8
1824:10.3390/molecules24081583
1674:10.1101/2024.03.29.587333
1424:Animal models in research
948:Mitochondrial dysfunction
798:is the main component of
769:dementia with Lewy bodies
635:electronic health records
566:Creutzfeldt–Jakob disease
561:Creutzfeldt-Jakob disease
555:Creutzfeldt–Jakob disease
381:cortices. The striatum's
240:amyloid precursor protein
89:neurodegenerative disease
69:
56:
47:
43:Neurodegenerative disease
42:
6781:Brain–computer interface
6730:Neuromorphic engineering
6655:Educational neuroscience
6562:Nutritional neuroscience
6467:Clinical neurophysiology
6363:Integrative neuroscience
6209:Progressive bulbar palsy
6000:Intracranial hypotension
5983:Choroid plexus papilloma
5397:Herpesviral encephalitis
4694:Progress in Neurobiology
3612:Nature Reviews Neurology
2928:Crossman AR (May 2000).
2323:Progress in Neurobiology
1432:), investigators employ
841:Intracellular mechanisms
588:
6592:Behavioral neuroscience
5721:Frontotemporal dementia
5407:Encephalitis lethargica
5185:10.1126/science.2111583
3969:10.15252/embj.201695810
3725:10.7748/ldp.22.5.22.s16
3003:10.1126/science.aax3338
2611:10.1074/jbc.M110.139576
2094:10.1007/12013.1559-0283
1478:doctor, is prescribing
1352:by trasglutaminases by
1340:have been proved to be
1260:, in a reaction termed
1031:reactive oxygen species
962:Reactive oxygen species
800:neurofibrillary tangles
773:multiple system atrophy
709:spinocerebellar ataxias
228:neurofibrillary tangles
125:multiple system atrophy
6929:Neurological disorders
6587:Affective neuroscience
6368:Molecular neuroscience
6323:Behavioral epigenetics
6026:Hepatic encephalopathy
4358:10.1074/jbc.R800041200
4307:10.1074/jbc.R800067200
4256:10.1074/jbc.R800065200
3518:Julien JP (May 2007).
1724:10.3390/antiox10111845
1511:Prevention of dementia
1217:Cytoplasmic (type III)
966:glutathione peroxidase
630:
497:Fused in Sarcoma (FUS)
435:central nervous system
175:
32:Experimental Neurology
6924:Clinical neuroscience
6650:Cultural neuroscience
6645:Consumer neuroscience
6487:Neurogastroenterology
6343:Cellular neuroscience
6084:Ataxia–telangiectasia
6041:Static encephalopathy
5764:Mitochondrial disease
5602:Spasmodic torticollis
5512:Basal ganglia disease
4288:Orr HT (March 2009).
2802:. In Purves D (ed.).
2649:Neurobiology of Aging
2243:(7S_Part_16): 800–1.
1668:: 2024.03.29.587333.
1484:neurological disorder
1363:augmented expression:
1130:Programmed cell death
1125:Programmed cell death
1092:Axonal swelling, and
1076:xeroderma pigmentosum
1068:ataxia telangiectasia
1041:. Damage to a cell's
982:Mitochondrial disease
974:mitochondrial fission
900:programmed cell death
880:ubiquitin–proteasome:
628:
593:Further information:
431:demyelinating disease
244:transmembrane protein
173:
6622:Sensory neuroscience
6462:Behavioral neurology
6433:Systems neuroscience
6031:Toxic encephalopathy
5737:Lewy bodies dementia
4547:10.1155/2017/2525967
3229:10.1093/brain/awn080
2716:10.1155/2020/7548394
2196:10.2147/PGPM.S284615
1603:10.3390/ijms23031851
1384:in these structures:
1201:Autophagic (type II)
1132:(PCD) is death of a
1121:is often triggered.
705:Huntington's disease
443:myelin basic protein
363:medium spiny neurons
348:Huntington's disease
343:Huntington's disease
337:Huntington's disease
121:Huntington's disease
6765:Social neuroscience
6665:Global neurosurgery
6542:Neurorehabilitation
6512:Neuro-ophthalmology
6497:Neurointensive care
6328:Behavioral genetics
6079:Friedreich's ataxia
5462:Meningoencephalitis
5402:Limbic encephalitis
5264:. January 12, 2024.
5177:1990Sci...248.1122E
4193:2008Natur.452..707T
4070:2023Natur.614...18K
3848:10.1038/nature05292
3840:2006Natur.443..787L
3674:(23): e2436–e2440.
3524:Nature Neuroscience
3418:Nature Neuroscience
3369:Nature Neuroscience
3274:2020NatSR..1015661M
2995:2020Sci...369..787B
2870:Nature Neuroscience
2703:Parkinson's Disease
2082:"Journal home page"
1989:10.1038/nature05293
1981:2006Natur.443..796B
1921:10.1038/nature05291
1913:2006Natur.443..780R
1410:abnormal structures
1390:(the result of the
1287:Alzheimer's disease
1060:Parkinson's disease
1056:Alzheimer's disease
793:hyperphosphorylated
781:Alzheimer's disease
485:lower motor neurons
481:upper motor neurons
447:proteolipid protein
398:polyglutamine tract
277:Parkinson's disease
272:Parkinson's disease
266:Parkinson's disease
211:, and parts of the
185:Alzheimer's disease
180:Alzheimer's disease
166:Alzheimer's disease
117:Alzheimer's disease
113:Parkinson's disease
63:Alzheimer's disease
6841:Neuroimmune system
6735:Neurophenomenology
6675:Neural engineering
6398:Neuroendocrinology
6378:Neural engineering
6115:Pseudobulbar palsy
5853:Status epilepticus
5804:Multiple sclerosis
5641:Myoclonic epilepsy
5504:movement disorders
5450:Acute disseminated
5392:Viral encephalitis
5014:10.1007/BF03256900
3537:10.1038/nn0507-535
3322:Muscle & Nerve
3262:Scientific Reports
2934:Journal of Anatomy
2755:Movement Disorders
2535:HHMI Research News
2501:Revue Neurologique
2378:10.1042/BST0330335
1141:Apoptosis (type I)
1107:cytoplasmic dynein
1052:oculomotor apraxia
919:neurodegeneration.
828:main component of
751:misfolded proteins
731:Protein misfolding
696:), and fruit fly (
631:
427:Multiple sclerosis
422:Multiple sclerosis
416:Multiple sclerosis
383:subthalamic nuclei
352:autosomal dominant
323:glucocerebrosidase
294:, a region of the
176:
158:Specific disorders
109:multiple sclerosis
6911:
6910:
6760:Paleoneurobiology
6695:Neuroepistemology
6670:Neuroanthropology
6636:Interdisciplinary
6522:Neuropharmacology
6482:Neuroepidemiology
6253:
6252:
6249:
6248:
6245:
6244:
6049:
6048:
5954:
5953:
5893:Template:Headache
5859:Template:Epilepsy
5780:
5779:
5754:Vascular dementia
5597:Status dystonicus
5470:
5469:
5445:Encephalomyelitis
5323:
5322:
4743:Annu Rev Neurosci
4594:(Pt A): 163–176.
4016:(7): 1086–93.e2.
3888:978-0-12-092860-6
3751:www.ninds.nih.gov
3575:www.ninds.nih.gov
3334:10.1002/mus.26764
3125:978-0-7234-3748-2
3091:www.ninds.nih.gov
2989:(6505): 787–793.
2815:978-0-87893-742-4
2767:10.1002/mds.26942
2655:: 209.e1–209.e7.
2155:978-1-4008-4846-1
2123:(Suppl 9): 7–10.
2067:978-1-4020-9434-7
2035:978-92-4-003324-5
1975:(7113): 796–802.
1774:10.1111/imm.12922
1640:www.ninds.nih.gov
1346:transglutaminases
1271:Transglutaminase
1236:Transglutaminases
1072:Cockayne syndrome
1048:repair mechanisms
666:genetic mutations
97:neurodegeneration
85:
84:
37:Medical condition
16:(Redirected from
6946:
6899:
6898:
6887:
6886:
6801:Detection theory
6685:Neurocriminology
6612:Neurolinguistics
6527:Neuroprosthetics
6445:
6408:Neuroinformatics
6358:Imaging genetics
6280:
6273:
6266:
6257:
6256:
6067:
6066:
6058:
6057:
6016:Brain herniation
5827:
5826:
5667:Intention tremor
5662:Essential tremor
5527:Postencephalitic
5495:
5494:
5486:
5485:
5375:
5374:
5356:Diseases of the
5350:
5343:
5336:
5327:
5326:
5273:
5272:
5266:
5265:
5254:
5248:
5247:
5237:
5227:
5203:
5197:
5196:
5171:(4959): 1122–4.
5160:
5154:
5153:
5117:
5111:
5110:
5100:
5068:
5062:
5061:
5024:
5018:
5017:
4997:
4991:
4986:
4980:
4979:
4943:
4920:
4919:
4909:
4898:10.1172/JCI26321
4877:
4871:
4870:
4834:
4828:
4827:
4817:
4807:
4783:
4777:
4776:
4766:
4734:
4728:
4727:
4717:
4685:
4674:
4673:
4663:
4631:
4622:
4621:
4611:
4579:
4570:
4569:
4559:
4549:
4525:
4519:
4518:
4490:
4484:
4483:
4473:
4463:
4439:
4433:
4432:
4422:
4412:
4387:
4381:
4380:
4370:
4360:
4336:
4330:
4329:
4319:
4309:
4285:
4279:
4278:
4268:
4258:
4234:
4223:
4222:
4204:
4172:
4163:
4162:
4152:
4142:
4118:
4112:
4109:
4107:
4105:
4055:
4045:
4035:
4025:
4000:
3991:
3990:
3980:
3957:The EMBO Journal
3948:
3942:
3941:
3931:
3899:
3893:
3892:
3874:
3868:
3867:
3834:(7113): 787–95.
3823:
3814:
3813:
3811:
3809:
3794:
3788:
3787:
3785:
3783:
3769:
3763:
3762:
3760:
3758:
3743:
3737:
3736:
3708:
3702:
3701:
3691:
3659:
3646:
3645:
3635:
3603:
3586:
3585:
3583:
3581:
3567:
3558:
3557:
3539:
3515:
3509:
3508:
3498:
3458:
3452:
3451:
3441:
3409:
3403:
3402:
3392:
3360:
3354:
3353:
3317:
3304:
3303:
3293:
3253:
3242:
3241:
3231:
3221:
3197:
3191:
3190:
3180:
3170:
3146:
3135:
3134:
3133:
3132:
3107:
3101:
3100:
3098:
3097:
3083:
3074:
3073:
3063:
3031:
3025:
3024:
3014:
2973:
2964:
2963:
2953:
2925:
2919:
2910:
2904:
2903:
2893:
2861:
2855:
2854:
2826:
2820:
2819:
2795:
2789:
2788:
2778:
2745:
2739:
2738:
2728:
2718:
2694:
2683:
2682:
2672:
2640:
2634:
2633:
2623:
2613:
2604:(42): 32486–93.
2589:
2578:
2577:
2549:
2538:
2531:
2525:
2524:
2496:
2490:
2489:
2471:
2439:
2433:
2432:
2396:
2390:
2389:
2361:
2355:
2354:
2318:
2312:
2311:
2301:
2269:
2263:
2262:
2252:
2228:
2219:
2218:
2208:
2198:
2174:
2168:
2167:
2139:
2133:
2132:
2112:
2106:
2105:
2078:
2072:
2071:
2053:
2047:
2046:
2044:
2042:
2027:
2017:
2011:
2010:
2000:
1960:
1941:
1940:
1896:
1873:
1872:
1870:
1868:
1853:
1847:
1846:
1836:
1826:
1802:
1796:
1795:
1785:
1753:
1747:
1746:
1736:
1726:
1702:
1696:
1695:
1685:
1657:
1651:
1650:
1648:
1646:
1632:
1626:
1625:
1615:
1605:
1581:
1575:
1574:
1564:
1530:
1406:isopeptide bonds
1402:Co-localization:
1392:transglutaminase
1388:isopeptide bonds
1386:The presence of
1382:isopeptide bonds
1371:transglutaminase
1361:Transglutaminase
1318:Transglutaminase
1258:isopeptide bonds
1231:Transglutaminase
1099:Axonal transport
1094:axonal spheroids
1088:Axonal transport
1082:Axonal transport
1011:oxidative damage
970:oxidative stress
609:oxidative stress
406:axonal transport
402:inclusion bodies
396:HD is caused by
292:substantia nigra
52:
40:
39:
21:
6954:
6953:
6949:
6948:
6947:
6945:
6944:
6943:
6914:
6913:
6912:
6907:
6875:
6861:Neurotechnology
6856:Neuroplasticity
6851:Neuromodulation
6846:Neuromanagement
6769:
6740:Neurophilosophy
6637:
6631:
6617:Neuropsychology
6578:
6571:
6532:Neuropsychiatry
6492:Neuroimmunology
6477:Neurocardiology
6453:
6446:
6437:
6428:Neurophysiology
6418:Neuromorphology
6373:Neural decoding
6314:
6307:
6289:
6284:
6254:
6241:
6167:Congenital DSMA
6088:
6045:
6004:
5950:
5938:Sleep disorders
5926:
5903:Cerebrovascular
5897:
5863:
5820:
5814:
5776:
5758:
5683:
5629:Choreoathetosis
5502:
5479:
5466:
5430:
5364:
5354:
5324:
5319:
5318:
5284:
5270:
5269:
5256:
5255:
5251:
5204:
5200:
5161:
5157:
5118:
5114:
5069:
5065:
5025:
5021:
4998:
4994:
4987:
4983:
4944:
4923:
4878:
4874:
4851:10.1038/nrn1100
4835:
4831:
4784:
4780:
4735:
4731:
4686:
4677:
4632:
4625:
4580:
4573:
4526:
4522:
4491:
4487:
4454:(17): 3159–80.
4440:
4436:
4388:
4384:
4337:
4333:
4286:
4282:
4235:
4226:
4202:10.1038/452707a
4187:(7188): 707–8.
4173:
4166:
4133:(8): e1010670.
4119:
4115:
4103:
4101:
4064:(7946): 18–19.
4001:
3994:
3963:(11): 1474–92.
3949:
3945:
3914:(10): a025130.
3900:
3896:
3889:
3875:
3871:
3824:
3817:
3807:
3805:
3795:
3791:
3781:
3779:
3771:
3770:
3766:
3756:
3754:
3745:
3744:
3740:
3709:
3705:
3660:
3649:
3604:
3589:
3579:
3577:
3569:
3568:
3561:
3516:
3512:
3459:
3455:
3410:
3406:
3361:
3357:
3318:
3307:
3254:
3245:
3219:10.1.1.328.2931
3198:
3194:
3147:
3138:
3130:
3128:
3126:
3108:
3104:
3095:
3093:
3085:
3084:
3077:
3032:
3028:
2974:
2967:
2926:
2922:
2911:
2907:
2876:(11): 1355–60.
2862:
2858:
2827:
2823:
2816:
2796:
2792:
2746:
2742:
2695:
2686:
2641:
2637:
2590:
2581:
2550:
2541:
2532:
2528:
2497:
2493:
2440:
2436:
2397:
2393:
2372:(Pt 2): 335–8.
2362:
2358:
2319:
2315:
2284:(27): 7212–21.
2270:
2266:
2229:
2222:
2175:
2171:
2156:
2140:
2136:
2113:
2109:
2080:
2079:
2075:
2068:
2054:
2050:
2040:
2038:
2036:
2025:
2019:
2018:
2014:
1961:
1944:
1907:(7113): 780–6.
1897:
1876:
1866:
1864:
1855:
1854:
1850:
1803:
1799:
1754:
1750:
1703:
1699:
1658:
1654:
1644:
1642:
1634:
1633:
1629:
1582:
1578:
1531:
1524:
1519:
1492:
1456:
1430:palliative care
1426:
1418:
1334:alpha-synuclein
1309:disease, it is
1303:alpha-synuclein
1301:disease, it is
1233:
1219:
1203:
1176:Golgi apparatus
1143:
1127:
1113:, cargoes, and
1090:
1084:
1027:
950:
935:
933:Membrane damage
848:
843:
777:amyloid plaques
759:alpha-synuclein
739:
733:
717:
662:
652:
647:
623:
597:
591:
586:
563:
557:
530:
524:
470:
464:
424:
418:
387:globus pallidus
356:huntingtin gene
350:(HD) is a rare
345:
339:
300:alpha-synuclein
290:neurons in the
274:
268:
256:gamma secretase
224:amyloid plaques
217:cingulate gyrus
197:cerebral cortex
182:
168:
160:
38:
35:
28:
23:
22:
15:
12:
11:
5:
6952:
6942:
6941:
6936:
6931:
6926:
6909:
6908:
6906:
6905:
6893:
6880:
6877:
6876:
6874:
6873:
6871:Self-awareness
6868:
6863:
6858:
6853:
6848:
6843:
6838:
6833:
6828:
6826:Neurodiversity
6823:
6818:
6813:
6808:
6803:
6798:
6793:
6788:
6783:
6777:
6775:
6771:
6770:
6768:
6767:
6762:
6757:
6752:
6747:
6742:
6737:
6732:
6727:
6725:Neuromarketing
6722:
6717:
6712:
6707:
6702:
6700:Neuroesthetics
6697:
6692:
6690:Neuroeconomics
6687:
6682:
6677:
6672:
6667:
6662:
6657:
6652:
6647:
6641:
6639:
6633:
6632:
6630:
6629:
6624:
6619:
6614:
6609:
6604:
6599:
6594:
6589:
6583:
6581:
6573:
6572:
6570:
6569:
6564:
6559:
6554:
6549:
6544:
6539:
6537:Neuroradiology
6534:
6529:
6524:
6519:
6517:Neuropathology
6514:
6509:
6507:Neuro-oncology
6504:
6499:
6494:
6489:
6484:
6479:
6474:
6469:
6464:
6458:
6456:
6448:
6447:
6440:
6438:
6436:
6435:
6430:
6425:
6420:
6415:
6410:
6405:
6400:
6395:
6393:Neurochemistry
6390:
6385:
6380:
6375:
6370:
6365:
6360:
6355:
6350:
6345:
6340:
6335:
6330:
6325:
6319:
6317:
6309:
6308:
6306:
6305:
6300:
6294:
6291:
6290:
6283:
6282:
6275:
6268:
6260:
6251:
6250:
6247:
6246:
6243:
6242:
6240:
6239:
6238:
6237:
6226:
6225:
6224:
6223:
6222:
6221:
6216:
6206:
6201:
6200:
6199:
6194:
6189:
6188:
6187:
6182:
6177:
6169:
6164:
6159:
6154:
6149:
6139:
6125:
6124:
6123:
6122:
6117:
6112:
6098:
6096:
6090:
6089:
6087:
6086:
6081:
6075:
6073:
6064:
6055:
6051:
6050:
6047:
6046:
6044:
6043:
6038:
6033:
6028:
6023:
6018:
6012:
6010:
6006:
6005:
6003:
6002:
5997:
5995:Cerebral edema
5992:
5991:
5990:
5985:
5980:
5975:
5964:
5962:
5956:
5955:
5952:
5951:
5949:
5948:
5947:
5946:
5944:Template:Sleep
5934:
5932:
5928:
5927:
5925:
5924:
5918:
5913:
5907:
5905:
5899:
5898:
5896:
5895:
5889:
5884:
5879:
5873:
5871:
5865:
5864:
5862:
5861:
5855:
5850:
5845:
5839:
5837:
5824:
5816:
5815:
5813:
5812:
5806:
5801:
5796:
5790:
5788:
5782:
5781:
5778:
5777:
5775:
5774:
5772:Leigh syndrome
5768:
5766:
5760:
5759:
5757:
5756:
5745:
5744:
5739:
5734:
5733:
5732:
5718:
5717:
5716:
5711:
5710:
5709:
5693:
5691:
5685:
5684:
5682:
5681:
5676:
5671:
5670:
5669:
5664:
5653:
5652:
5651:
5650:
5645:
5644:
5643:
5633:
5632:
5631:
5621:
5616:
5615:
5614:
5609:
5604:
5599:
5583:
5582:
5581:
5580:
5575:
5570:
5565:
5560:
5559:
5558:
5548:
5547:
5546:
5536:
5535:
5534:
5529:
5524:
5508:
5506:
5499:Extrapyramidal
5492:
5483:
5481:encephalopathy
5472:
5471:
5468:
5467:
5465:
5464:
5459:
5454:
5453:
5452:
5441:
5439:
5432:
5431:
5429:
5428:
5427:
5426:
5416:
5411:
5410:
5409:
5404:
5399:
5394:
5383:
5381:
5372:
5366:
5365:
5358:nervous system
5353:
5352:
5345:
5338:
5330:
5321:
5320:
5317:
5316:
5305:
5285:
5280:
5279:
5277:
5276:Classification
5268:
5267:
5249:
5218:(9): a006387.
5198:
5155:
5112:
5063:
5019:
4992:
4981:
4921:
4892:(10): 2610–7.
4872:
4829:
4778:
4729:
4700:(2): 166–200.
4675:
4646:(2): 266–282.
4623:
4571:
4520:
4485:
4434:
4397:Genome Biology
4382:
4351:(12): 7425–9.
4331:
4280:
4249:(12): 7431–5.
4224:
4164:
4127:PLOS Pathogens
4113:
4111:
4110:
3992:
3943:
3894:
3887:
3869:
3815:
3789:
3764:
3738:
3703:
3647:
3618:(3): 161–178.
3587:
3559:
3510:
3473:(6): a020628.
3453:
3430:10.1038/nn1885
3404:
3381:10.1038/nn1876
3355:
3328:(4): 508–511.
3305:
3243:
3212:(6): 1464–77.
3192:
3136:
3124:
3102:
3075:
3026:
2965:
2920:
2905:
2882:10.1038/nn1988
2856:
2821:
2814:
2790:
2761:(5): 739–749.
2740:
2684:
2635:
2579:
2539:
2537:June 22, 2006.
2526:
2491:
2454:(5): 511–524.
2434:
2407:(11): 1984–9.
2391:
2356:
2313:
2264:
2220:
2169:
2154:
2134:
2107:
2073:
2066:
2048:
2034:
2012:
1942:
1874:
1863:. 17 July 2014
1848:
1797:
1768:(2): 204–219.
1748:
1697:
1652:
1627:
1576:
1521:
1520:
1518:
1515:
1514:
1513:
1508:
1503:
1498:
1491:
1488:
1455:
1452:
1425:
1422:
1417:
1414:
1376:is increased.
1354:covalent bonds
1262:transamidation
1254:covalent bonds
1232:
1229:
1218:
1215:
1202:
1199:
1180:
1179:
1161:
1142:
1139:
1126:
1123:
1086:Main article:
1083:
1080:
1026:
1023:
949:
946:
934:
931:
930:
929:
928:
927:
920:
916:macroautophagy
905:macroautophagy
893:
892:
891:
873:
872:
869:
866:
861:nucleus, e.g.
859:
847:
844:
842:
839:
838:
837:
830:prion diseases
820:
811:
784:
737:Stress granule
732:
729:
716:
713:
651:
648:
646:
643:
633:A study using
622:
619:
590:
587:
585:
582:
559:Main article:
556:
553:
533:Batten disease
528:Batten disease
526:Main article:
523:
522:Batten disease
520:
466:Main article:
463:
460:
456:HLA-DRB1*15:01
420:Main article:
417:
414:
341:Main article:
338:
335:
331:gut microbiome
270:Main article:
267:
264:
260:beta secretase
213:frontal cortex
178:Main article:
167:
164:
159:
156:
133:prion diseases
83:
82:
73:
67:
66:
54:
53:
45:
44:
36:
26:
9:
6:
4:
3:
2:
6951:
6940:
6937:
6935:
6932:
6930:
6927:
6925:
6922:
6921:
6919:
6904:
6903:
6894:
6892:
6891:
6882:
6881:
6878:
6872:
6869:
6867:
6864:
6862:
6859:
6857:
6854:
6852:
6849:
6847:
6844:
6842:
6839:
6837:
6834:
6832:
6829:
6827:
6824:
6822:
6819:
6817:
6814:
6812:
6809:
6807:
6804:
6802:
6799:
6797:
6794:
6792:
6789:
6787:
6784:
6782:
6779:
6778:
6776:
6772:
6766:
6763:
6761:
6758:
6756:
6755:Neurotheology
6753:
6751:
6750:Neurorobotics
6748:
6746:
6745:Neuropolitics
6743:
6741:
6738:
6736:
6733:
6731:
6728:
6726:
6723:
6721:
6718:
6716:
6713:
6711:
6710:Neuroethology
6708:
6706:
6703:
6701:
6698:
6696:
6693:
6691:
6688:
6686:
6683:
6681:
6678:
6676:
6673:
6671:
6668:
6666:
6663:
6661:
6658:
6656:
6653:
6651:
6648:
6646:
6643:
6642:
6640:
6634:
6628:
6625:
6623:
6620:
6618:
6615:
6613:
6610:
6608:
6607:Motor control
6605:
6603:
6600:
6598:
6597:Chronobiology
6595:
6593:
6590:
6588:
6585:
6584:
6582:
6580:
6574:
6568:
6565:
6563:
6560:
6558:
6557:Neurovirology
6555:
6553:
6550:
6548:
6545:
6543:
6540:
6538:
6535:
6533:
6530:
6528:
6525:
6523:
6520:
6518:
6515:
6513:
6510:
6508:
6505:
6503:
6500:
6498:
6495:
6493:
6490:
6488:
6485:
6483:
6480:
6478:
6475:
6473:
6470:
6468:
6465:
6463:
6460:
6459:
6457:
6455:
6449:
6444:
6434:
6431:
6429:
6426:
6424:
6421:
6419:
6416:
6414:
6411:
6409:
6406:
6404:
6403:Neurogenetics
6401:
6399:
6396:
6394:
6391:
6389:
6386:
6384:
6381:
6379:
6376:
6374:
6371:
6369:
6366:
6364:
6361:
6359:
6356:
6354:
6351:
6349:
6346:
6344:
6341:
6339:
6338:Brain-reading
6336:
6334:
6333:Brain mapping
6331:
6329:
6326:
6324:
6321:
6320:
6318:
6316:
6310:
6304:
6301:
6299:
6296:
6295:
6292:
6288:
6281:
6276:
6274:
6269:
6267:
6262:
6261:
6258:
6236:
6233:
6232:
6231:
6228:
6227:
6220:
6217:
6215:
6212:
6211:
6210:
6207:
6205:
6202:
6198:
6195:
6193:
6190:
6186:
6183:
6181:
6178:
6176:
6173:
6172:
6170:
6168:
6165:
6163:
6160:
6158:
6155:
6153:
6150:
6148:
6145:
6144:
6143:
6140:
6138:
6135:
6134:
6133:
6131:
6127:
6126:
6121:
6118:
6116:
6113:
6111:
6108:
6107:
6106:
6104:
6100:
6099:
6097:
6095:
6091:
6085:
6082:
6080:
6077:
6076:
6074:
6072:
6068:
6065:
6063:
6059:
6056:
6052:
6042:
6039:
6037:
6034:
6032:
6029:
6027:
6024:
6022:
6021:Reye syndrome
6019:
6017:
6014:
6013:
6011:
6007:
6001:
5998:
5996:
5993:
5989:
5986:
5984:
5981:
5979:
5976:
5974:
5973:Hydrocephalus
5971:
5970:
5969:
5966:
5965:
5963:
5961:
5957:
5945:
5941:
5940:
5939:
5936:
5935:
5933:
5929:
5923:
5919:
5917:
5914:
5912:
5909:
5908:
5906:
5904:
5900:
5894:
5890:
5888:
5885:
5883:
5880:
5878:
5875:
5874:
5872:
5870:
5866:
5860:
5856:
5854:
5851:
5849:
5846:
5844:
5841:
5840:
5838:
5836:
5832:
5828:
5825:
5823:
5817:
5811:
5807:
5805:
5802:
5800:
5797:
5795:
5792:
5791:
5789:
5787:
5786:Demyelinating
5783:
5773:
5770:
5769:
5767:
5765:
5761:
5755:
5752:
5751:
5750:
5749:
5743:
5740:
5738:
5735:
5731:
5728:
5727:
5726:
5722:
5719:
5715:
5712:
5708:
5705:
5704:
5703:
5700:
5699:
5698:
5695:
5694:
5692:
5690:
5686:
5680:
5677:
5675:
5674:Restless legs
5672:
5668:
5665:
5663:
5660:
5659:
5658:
5655:
5654:
5649:
5646:
5642:
5639:
5638:
5637:
5634:
5630:
5627:
5626:
5625:
5622:
5620:
5617:
5613:
5612:Blepharospasm
5610:
5608:
5605:
5603:
5600:
5598:
5595:
5594:
5593:
5590:
5589:
5588:
5585:
5584:
5579:
5576:
5574:
5571:
5569:
5568:Hemiballismus
5566:
5564:
5561:
5557:
5554:
5553:
5552:
5549:
5545:
5542:
5541:
5540:
5537:
5533:
5530:
5528:
5525:
5523:
5520:
5519:
5518:
5515:
5514:
5513:
5510:
5509:
5507:
5505:
5500:
5496:
5493:
5491:
5487:
5484:
5482:
5477:
5473:
5463:
5460:
5458:
5455:
5451:
5448:
5447:
5446:
5443:
5442:
5440:
5438:
5433:
5425:
5422:
5421:
5420:
5419:Brain abscess
5417:
5415:
5412:
5408:
5405:
5403:
5400:
5398:
5395:
5393:
5390:
5389:
5388:
5385:
5384:
5382:
5380:
5376:
5373:
5371:
5367:
5363:
5359:
5351:
5346:
5344:
5339:
5337:
5332:
5331:
5328:
5315:
5311:
5310:
5306:
5304:
5300:
5296:
5295:
5291:
5287:
5286:
5283:
5278:
5274:
5263:
5259:
5253:
5245:
5241:
5236:
5231:
5226:
5221:
5217:
5213:
5209:
5202:
5194:
5190:
5186:
5182:
5178:
5174:
5170:
5166:
5159:
5151:
5147:
5143:
5139:
5135:
5131:
5127:
5123:
5116:
5108:
5104:
5099:
5094:
5090:
5086:
5082:
5078:
5074:
5067:
5059:
5055:
5051:
5047:
5043:
5039:
5036:(5): 332–44.
5035:
5031:
5023:
5015:
5011:
5007:
5003:
4996:
4990:
4985:
4977:
4973:
4969:
4965:
4961:
4957:
4953:
4949:
4942:
4940:
4938:
4936:
4934:
4932:
4930:
4928:
4926:
4917:
4913:
4908:
4903:
4899:
4895:
4891:
4887:
4883:
4876:
4868:
4864:
4860:
4856:
4852:
4848:
4845:(5): 365–75.
4844:
4840:
4833:
4825:
4821:
4816:
4811:
4806:
4801:
4797:
4793:
4792:PLOS Genetics
4789:
4782:
4774:
4770:
4765:
4760:
4756:
4752:
4748:
4744:
4740:
4733:
4725:
4721:
4716:
4711:
4707:
4703:
4699:
4695:
4691:
4684:
4682:
4680:
4671:
4667:
4662:
4657:
4653:
4649:
4645:
4641:
4637:
4630:
4628:
4619:
4615:
4610:
4605:
4601:
4597:
4593:
4589:
4585:
4578:
4576:
4567:
4563:
4558:
4553:
4548:
4543:
4539:
4535:
4531:
4524:
4516:
4512:
4508:
4504:
4500:
4496:
4489:
4481:
4477:
4472:
4467:
4462:
4457:
4453:
4449:
4445:
4438:
4430:
4426:
4421:
4416:
4411:
4406:
4402:
4398:
4394:
4386:
4378:
4374:
4369:
4364:
4359:
4354:
4350:
4346:
4342:
4335:
4327:
4323:
4318:
4313:
4308:
4303:
4299:
4295:
4291:
4284:
4276:
4272:
4267:
4262:
4257:
4252:
4248:
4244:
4240:
4233:
4231:
4229:
4220:
4216:
4212:
4208:
4203:
4198:
4194:
4190:
4186:
4182:
4178:
4171:
4169:
4160:
4156:
4151:
4146:
4141:
4136:
4132:
4128:
4124:
4117:
4099:
4095:
4091:
4087:
4083:
4079:
4075:
4071:
4067:
4063:
4059:
4054:
4047:
4046:
4043:
4039:
4034:
4029:
4024:
4019:
4015:
4011:
4007:
3999:
3997:
3988:
3984:
3979:
3974:
3970:
3966:
3962:
3958:
3954:
3947:
3939:
3935:
3930:
3925:
3921:
3917:
3913:
3909:
3905:
3898:
3890:
3884:
3880:
3873:
3865:
3861:
3857:
3853:
3849:
3845:
3841:
3837:
3833:
3829:
3822:
3820:
3804:
3800:
3793:
3778:
3774:
3768:
3752:
3748:
3742:
3734:
3730:
3726:
3722:
3718:
3714:
3707:
3699:
3695:
3690:
3685:
3681:
3677:
3673:
3669:
3665:
3658:
3656:
3654:
3652:
3643:
3639:
3634:
3629:
3625:
3621:
3617:
3613:
3609:
3602:
3600:
3598:
3596:
3594:
3592:
3576:
3572:
3566:
3564:
3555:
3551:
3547:
3543:
3538:
3533:
3529:
3525:
3521:
3514:
3506:
3502:
3497:
3492:
3488:
3484:
3480:
3476:
3472:
3468:
3464:
3457:
3449:
3445:
3440:
3435:
3431:
3427:
3424:(5): 608–14.
3423:
3419:
3415:
3408:
3400:
3396:
3391:
3386:
3382:
3378:
3375:(5): 615–22.
3374:
3370:
3366:
3359:
3351:
3347:
3343:
3339:
3335:
3331:
3327:
3323:
3316:
3314:
3312:
3310:
3301:
3297:
3292:
3287:
3283:
3279:
3275:
3271:
3267:
3263:
3259:
3252:
3250:
3248:
3239:
3235:
3230:
3225:
3220:
3215:
3211:
3207:
3203:
3196:
3188:
3184:
3179:
3174:
3169:
3164:
3160:
3156:
3155:F1000Research
3152:
3145:
3143:
3141:
3127:
3121:
3117:
3113:
3106:
3092:
3088:
3082:
3080:
3071:
3067:
3062:
3057:
3053:
3049:
3046:(8): 378–85.
3045:
3041:
3037:
3030:
3022:
3018:
3013:
3008:
3004:
3000:
2996:
2992:
2988:
2984:
2980:
2972:
2970:
2961:
2957:
2952:
2947:
2943:
2939:
2935:
2931:
2924:
2918:
2914:
2909:
2901:
2897:
2892:
2887:
2883:
2879:
2875:
2871:
2867:
2860:
2852:
2848:
2844:
2840:
2837:(3): 265–76.
2836:
2832:
2825:
2817:
2811:
2807:
2806:
2801:
2794:
2786:
2782:
2777:
2772:
2768:
2764:
2760:
2756:
2752:
2744:
2736:
2732:
2727:
2722:
2717:
2712:
2708:
2704:
2700:
2693:
2691:
2689:
2680:
2676:
2671:
2666:
2662:
2658:
2654:
2650:
2646:
2639:
2631:
2627:
2622:
2617:
2612:
2607:
2603:
2599:
2595:
2588:
2586:
2584:
2575:
2571:
2567:
2563:
2559:
2555:
2548:
2546:
2544:
2536:
2530:
2522:
2518:
2514:
2510:
2506:
2502:
2495:
2487:
2483:
2479:
2475:
2470:
2465:
2461:
2457:
2453:
2449:
2445:
2438:
2430:
2426:
2422:
2418:
2414:
2410:
2406:
2402:
2395:
2387:
2383:
2379:
2375:
2371:
2367:
2360:
2352:
2348:
2344:
2340:
2336:
2332:
2328:
2324:
2317:
2309:
2305:
2300:
2295:
2291:
2287:
2283:
2279:
2275:
2268:
2260:
2256:
2251:
2246:
2242:
2238:
2234:
2227:
2225:
2216:
2212:
2207:
2202:
2197:
2192:
2188:
2184:
2180:
2173:
2165:
2161:
2157:
2151:
2147:
2146:
2138:
2130:
2126:
2122:
2118:
2111:
2103:
2099:
2095:
2091:
2087:
2083:
2077:
2069:
2063:
2059:
2052:
2037:
2031:
2024:
2023:
2016:
2008:
2004:
1999:
1994:
1990:
1986:
1982:
1978:
1974:
1970:
1966:
1959:
1957:
1955:
1953:
1951:
1949:
1947:
1938:
1934:
1930:
1926:
1922:
1918:
1914:
1910:
1906:
1902:
1895:
1893:
1891:
1889:
1887:
1885:
1883:
1881:
1879:
1862:
1861:JPND Research
1858:
1852:
1844:
1840:
1835:
1830:
1825:
1820:
1816:
1812:
1808:
1801:
1793:
1789:
1784:
1779:
1775:
1771:
1767:
1763:
1759:
1752:
1744:
1740:
1735:
1730:
1725:
1720:
1716:
1712:
1708:
1701:
1693:
1689:
1684:
1679:
1675:
1671:
1667:
1663:
1656:
1641:
1637:
1631:
1623:
1619:
1614:
1609:
1604:
1599:
1595:
1591:
1590:Int J Mol Sci
1587:
1580:
1572:
1568:
1563:
1558:
1554:
1550:
1546:
1542:
1541:
1536:
1529:
1527:
1522:
1512:
1509:
1507:
1504:
1502:
1501:JUNQ and IPOD
1499:
1497:
1494:
1493:
1487:
1485:
1481:
1477:
1472:
1468:
1465:
1464:immunotherapy
1460:
1451:
1449:
1443:
1440:
1435:
1434:animal models
1431:
1421:
1413:
1411:
1407:
1403:
1399:
1397:
1393:
1389:
1385:
1383:
1377:
1375:
1372:
1368:
1364:
1362:
1357:
1355:
1351:
1347:
1343:
1339:
1335:
1331:
1327:
1324:
1322:
1319:
1314:
1312:
1308:
1304:
1300:
1296:
1292:
1288:
1283:
1281:
1276:
1274:
1269:
1267:
1263:
1259:
1255:
1251:
1246:
1244:
1241:
1237:
1228:
1225:
1214:
1212:
1211:autophagosome
1208:
1198:
1196:
1192:
1188:
1184:
1177:
1173:
1169:
1165:
1162:
1159:
1155:
1152:
1151:
1150:
1147:
1138:
1135:
1131:
1122:
1120:
1116:
1112:
1108:
1104:
1100:
1095:
1089:
1079:
1077:
1073:
1069:
1065:
1061:
1057:
1053:
1049:
1044:
1040:
1036:
1032:
1022:
1020:
1016:
1015:transcription
1012:
1008:
1004:
1002:
998:
994:
990:
985:
983:
979:
975:
971:
967:
963:
959:
955:
945:
944:
941:
924:
921:
917:
914:
913:
911:
907:
906:
901:
897:
894:
889:
888:
885:
881:
878:
877:
876:
870:
867:
864:
860:
857:
856:
855:
853:
852:proteopathies
835:
831:
827:
825:
821:
818:
816:
812:
809:
805:
801:
797:
794:
791:
789:
785:
782:
778:
774:
770:
766:
762:
760:
756:
755:
754:
752:
748:
744:
743:proteopathies
738:
728:
726:
725:Braak staging
722:
712:
710:
706:
701:
699:
695:
691:
687:
681:
679:
675:
671:
667:
661:
657:
642:
640:
636:
627:
618:
615:
610:
606:
602:
596:
581:
579:
575:
571:
567:
562:
552:
550:
546:
542:
538:
534:
529:
519:
517:
516:motor neurons
513:
509:
504:
502:
498:
494:
490:
486:
482:
478:
474:
469:
459:
457:
452:
448:
444:
440:
436:
432:
428:
423:
413:
411:
407:
403:
399:
394:
392:
388:
384:
380:
376:
372:
368:
364:
360:
357:
353:
349:
344:
334:
332:
328:
324:
320:
315:
313:
309:
305:
301:
297:
293:
289:
284:
282:
278:
273:
263:
261:
257:
253:
249:
245:
241:
237:
233:
229:
225:
220:
218:
214:
210:
209:parietal lobe
206:
205:temporal lobe
202:
198:
194:
190:
186:
181:
172:
163:
155:
153:
148:
146:
142:
141:proteinopathy
138:
134:
130:
126:
122:
118:
114:
110:
106:
102:
98:
94:
90:
81:
77:
74:
72:
68:
64:
60:
55:
51:
46:
41:
33:
19:
6900:
6888:
6836:Neuroimaging
6831:Neurogenesis
6815:
6715:Neurohistory
6680:Neurobiotics
6579:neuroscience
6547:Neurosurgery
6472:Epileptology
6454:neuroscience
6423:Neurophysics
6413:Neurometrics
6388:Neurobiology
6383:Neuroanatomy
6353:Connectomics
6287:Neuroscience
6229:
6128:
6101:
6062:Degenerative
6061:
5799:Inflammatory
5746:
5679:Stiff-person
5517:Parkinsonism
5490:Degenerative
5489:
5387:Encephalitis
5370:Inflammation
5360:, primarily
5307:
5288:
5252:
5215:
5211:
5201:
5168:
5164:
5158:
5128:(4): 642–9.
5125:
5121:
5115:
5080:
5076:
5066:
5033:
5029:
5022:
5008:(2): 113–5.
5005:
5001:
4995:
4984:
4954:(2): 653–8.
4951:
4947:
4889:
4885:
4875:
4842:
4838:
4832:
4798:(10): e135.
4795:
4791:
4781:
4746:
4742:
4732:
4697:
4693:
4643:
4639:
4591:
4587:
4537:
4533:
4523:
4498:
4494:
4488:
4451:
4447:
4437:
4400:
4396:
4385:
4348:
4344:
4334:
4300:(12): 7405.
4297:
4293:
4283:
4246:
4242:
4184:
4180:
4130:
4126:
4116:
4102:. Retrieved
4061:
4057:
4013:
4009:
3960:
3956:
3946:
3911:
3907:
3897:
3878:
3872:
3831:
3827:
3806:. Retrieved
3802:
3792:
3780:. Retrieved
3776:
3767:
3755:. Retrieved
3750:
3741:
3716:
3712:
3706:
3671:
3667:
3615:
3611:
3578:. Retrieved
3574:
3530:(5): 535–7.
3527:
3523:
3513:
3470:
3466:
3456:
3421:
3417:
3407:
3372:
3368:
3358:
3325:
3321:
3268:(1): 15661.
3265:
3261:
3209:
3205:
3195:
3158:
3154:
3129:, retrieved
3115:
3105:
3094:. Retrieved
3090:
3043:
3039:
3029:
2986:
2982:
2933:
2923:
2908:
2873:
2869:
2859:
2834:
2830:
2824:
2805:Neuroscience
2804:
2793:
2758:
2754:
2743:
2706:
2702:
2652:
2648:
2638:
2601:
2597:
2557:
2553:
2529:
2507:(1): 14–26.
2504:
2500:
2494:
2451:
2447:
2437:
2404:
2400:
2394:
2369:
2365:
2359:
2326:
2322:
2316:
2281:
2277:
2267:
2240:
2236:
2186:
2182:
2172:
2144:
2137:
2120:
2116:
2110:
2088:. Springer.
2085:
2076:
2057:
2051:
2039:. Retrieved
2021:
2015:
1972:
1968:
1904:
1900:
1865:. Retrieved
1860:
1851:
1814:
1810:
1800:
1765:
1761:
1751:
1717:(11): 1845.
1714:
1711:Antioxidants
1710:
1700:
1665:
1655:
1643:. Retrieved
1639:
1630:
1593:
1589:
1579:
1544:
1538:
1480:table tennis
1473:
1469:
1462:The goal of
1461:
1457:
1448:amyloid-beta
1444:
1427:
1419:
1401:
1400:
1380:Presence of
1379:
1378:
1359:
1358:
1326:Amyloid-beta
1316:
1315:
1307:Huntington's
1291:amyloid-beta
1289:, these are
1284:
1277:
1270:
1266:crosslinking
1247:
1243:ubiquitously
1234:
1220:
1207:phagocytosis
1204:
1194:
1190:
1181:
1168:cytochrome c
1163:
1153:
1144:
1128:
1115:mitochondria
1111:microtubules
1091:
1028:
1005:
997:Huntington's
986:
954:cytochrome c
951:
936:
922:
915:
909:
903:
895:
879:
874:
849:
822:
815:amyloid beta
813:
786:
757:
740:
718:
702:
697:
693:
682:
663:
632:
598:
584:Risk factors
564:
531:
507:
505:
477:Lou Gehrig's
471:
455:
425:
395:
367:astrogliosis
358:
346:
316:
288:dopaminergic
285:
281:bradykinesia
275:
236:amyloid beta
221:
183:
161:
149:
96:
88:
86:
6705:Neuroethics
6552:Neurotology
6214:Fazio–Londe
6054:Both/either
5848:Generalised
5707:Early-onset
5702:Alzheimer's
5437:spinal cord
4948:Amino Acids
4540:: 2525967.
4104:15 February
3777:Mayo Clinic
3580:30 November
2913:Purves 2001
2818:. NBK10847.
2329:(1): 1–32.
2189:: 631–653.
1867:February 7,
1817:(8): 1583.
1596:(3): 1851.
1408:with these
1299:Parkinson's
1019:antioxidant
993:Parkinson's
989:Alzheimer's
926:substrates.
804:Pick bodies
796:tau protein
765:Lewy bodies
747:aggregation
715:Epigenetics
607:as well as
595:Aging brain
568:(CJD) is a
483:(UMNs) and
451:macrophages
327:tau protein
325:(GBA), and
308:Lewy bodies
145:therapeutic
137:subcellular
129:tauopathies
6934:Senescence
6918:Categories
6866:Neurotoxin
6567:Psychiatry
5822:paroxysmal
5794:Autoimmune
5587:Dyskinesia
5457:Meningitis
5435:Brain and
5083:: 175–93.
4749:: 245–67.
4501:: 91–123.
3131:2020-12-07
3096:2020-11-30
2560:: 151–73.
2041:14 October
1762:Immunology
1517:References
1416:Management
1367:expression
1342:substrates
1338:huntingtin
1321:substrates
1311:huntingtin
1238:are human
1191:initiators
1187:amino acid
1160:-8 or -10.
1035:DNA damage
1025:DNA damage
898:a form of
735:See also:
698:Drosophila
694:C. elegans
654:See also:
645:Mechanisms
639:UK Biobank
621:Infections
614:DNA damage
512:astrocytes
80:Psychiatry
6811:Neurochip
6577:Cognitive
6502:Neurology
5819:Episodic/
5697:Tauopathy
5648:Akathisia
5636:Myoclonus
5619:Athetosis
5551:Tauopathy
5002:Pharm Med
4403:(1): 90.
4219:205037169
4094:256193462
3733:241832253
3719:(5): 22.
3668:Neurology
3487:1943-0264
3350:208186566
3214:CiteSeerX
2401:Neurology
2164:859536969
2102:1559-0283
1811:Molecules
1547:(4): 20.
1195:effectors
1146:Apoptosis
1021:defense.
956:from the
884:ubiquitin
806:found in
670:glutamine
539:known as
321:(LRRK2),
304:ubiquitin
242:(APP), a
76:Neurology
71:Specialty
6890:Category
6774:Concepts
6720:Neurolaw
6452:Clinical
6185:SMALED2B
6180:SMALED2A
5877:Migraine
5869:Headache
5835:epilepsy
5831:Seizures
5689:Dementia
5592:Dystonia
5262:Fox News
5244:22951439
5150:54334969
5142:15080893
5107:18352830
5058:39817779
5050:18790460
4976:19739739
4968:19960212
4916:16200193
4867:33018251
4859:12728264
4824:17069462
4773:20345246
4724:21550379
4670:25033177
4618:27663141
4566:28785371
4515:18333761
4480:29947927
4429:33771206
4377:18957430
4326:18957428
4275:18957429
4211:18401401
4159:35925897
4098:Archived
4086:36690772
4042:36669485
4033:10079561
3987:28438892
3938:26385091
3856:17051205
3808:31 March
3782:31 March
3757:31 March
3698:32300063
3642:30783219
3546:17453052
3505:25877220
3448:17435754
3399:17435755
3342:31743477
3300:32973334
3238:18490361
3187:31885862
3161:: 2100.
3070:23768628
3021:32675289
2960:10923984
2900:17965655
2851:18279698
2785:28195358
2735:33274040
2679:26601739
2630:20693280
2574:18558852
2521:26718594
2486:25739126
2478:15004691
2469:11138910
2429:25017332
2421:15184601
2386:15787600
2351:25376584
2343:12927332
2308:16822978
2259:54359937
2215:34093032
2129:12934968
2007:17051206
1929:17051204
1843:31013638
1792:29513402
1743:34829716
1692:38585882
1683:10996678
1622:35163773
1571:28716886
1490:See also
1224:necrosis
1183:Caspases
1158:caspases
882:protein
707:and the
690:nematode
650:Genetics
549:dementia
545:epilepsy
508:in vitro
379:temporal
371:striatum
296:midbrain
254:such as
232:peptides
193:synapses
152:dementia
6902:Commons
6315:science
6303:History
6298:Outline
6197:SMA-PME
6192:SMA-PCH
6175:SMALED1
5887:Tension
5882:Cluster
5607:Meige's
5424:Amoebic
5314:D019636
5235:3426815
5193:2111583
5173:Bibcode
5165:Science
5098:2561172
4907:1236695
4815:1626106
4764:5223592
4715:3123739
4661:5564444
4609:5316312
4557:5529664
4471:6063327
4420:8004462
4368:2658037
4317:2658033
4266:2658038
4189:Bibcode
4150:9352104
4066:Bibcode
3978:5452017
3929:4588127
3864:4421515
3836:Bibcode
3689:7455368
3633:6681450
3554:2987257
3496:4448607
3439:3139463
3390:3799799
3291:7518279
3270:Bibcode
3178:6915812
3061:3955166
3012:7859879
2991:Bibcode
2983:Science
2951:1468094
2891:3110080
2776:5469442
2726:7683170
2709:: 1–5.
2670:4688052
2621:2952250
2299:6673945
2206:8169052
1998:3970704
1977:Bibcode
1937:4411895
1909:Bibcode
1834:6514564
1783:5980185
1734:8614795
1666:bioRxiv
1645:12 July
1613:8837071
1562:5880171
1496:Amyloid
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