495:
If a patient does not meet this criterion of diagnosis, they must have a family history of NF2, and present with a unilateral vestibular schwannoma and other associated tumors (cranial meningioma, cranial nerve schwannoma, spinal meningioma, spinal ependymomas, peripheral nerve tumor, spinal schwannoma, subcutaneous tumor, skin plaque). This being said, more than half of all patients diagnosed with NF2 do not have a family history of the condition. Although it has yet to be included into clinical classification, peripheral neuropathy, or damage to the peripheral nerves, which often causes weakness, numbness and pain in the hands and feet, may also lead to a diagnosis of NF2. In children, NF2 can present with similar symptoms, but generally causes "visual disturbances (cataracts, hamartomas), skin tumors, mononeuropathhy (facial paresis, drop foot), symptomatic spinal cord tumors, or non-vestibular intracranial tumors".
381:
40:
68:
226:
487:
400:
638:
perception testing by Neff et al., they discovered that the use of cochlear implants with NF2 patients allowed significant improvement of hearing abilities. In testing of recognition of sentences of everyday speech, five out of six patients scored within the 90–100% range, and in testing of hearing in noise setting, four of six of the patients scored within the 83–96%. Additionally, all testing was done without lip-reading.
408:
84:– multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves. The types of tumors frequently associated with NF2 include vestibular schwannomas, meningiomas, and ependymomas. The main manifestation of the condition is the development of bilateral
597:
of the IAC without any attempt to remove the tumor. The bone overlying the acoustic nerve is removed, allowing the tumour to expand upward into the middle cranial fossa. In this way, pressure on the cochlear nerve is relieved, reducing the risk of further hearing loss from direct compression or obstruction of vascular supply to the nerve.
592:
Larger tumors can be treated by either the translabyrinthine approach or the retrosigmoid approach, depending upon the experience of the surgical team. With large tumors, the chance of hearing preservation is small with any approach. When hearing is already poor, the translabyrinthine approach may be
666:
Truncating mutations lead to smaller and non-functional protein products. Studies have shown that missense mutations and large deletions can both cause predominantly mild phenotypes. Phenotype is more variable in patients with splice-site mutations, and a milder disease in patients with mutations in
655:
NF2 is a life limiting condition. It is a rare genetic disorder that involves noncancerous tumors of the nerves that transmit balance and sound impulses from the inner ear to the brain. The prognosis is affected by early age onset, a higher number of meningiomas and schwannomas and having a decrease
494:
NF2 is a genetically transmitted condition. Diagnosis is most common in early adulthood (20–30 years); however, it can be diagnosed earlier. NF2 can be diagnosed due to the presence of a bilateral vestibular schwannoma, or an acoustic neuroma, which causes a hearing loss that may begin unilaterally.
596:
Auditory canal decompression is another surgical technique that can prolong usable hearing when a vestibular schwannoma has grown too large to remove without damage to the cochlear nerve. In the IAC (internal auditory canal) decompression, a middle fossa approach is employed to expose the bony roof
269:
The hearing loss caused by NF2 is gradual and results from the presences of bilateral cochleovestibular schwannomas, also known as acoustic neuromas, which damage to cochlear nerve causing hearing loss. Hearing loss may also result from benign tumors that grow on the vestibular and auditory nerves,
637:
A cochlear implant is an electronic device that is surgically implants to stimulate the cochlear nerve. Cochlear implants will work only when the cochleovestibular nerve (8th nerve) and the cochlea are still functioning. In a study done with open-set speech perception testing and closed-set speech
432:
Many people with NF2 were included in studies that were designed to compare disease type and progression with exact determination of the associated mutation. The goal of such comparisons of genotype and phenotype is to determine whether specific mutations cause respective combinations of symptoms.
326:
due to the lack of contact-mediated tumour suppression, mainly because of the cell:cell junction disruption, sufficient to result in the tumors characteristic of
Neurofibromatosis type II. Recent studies showed that besides its cytoskeletal and cytoplasmic functions Merlin also translocates to the
423:
of the nervus vestibularis, or vestibular schwannoma. The misnomer of acoustic neuroma is still often used. The vestibular schwannomas grow slowly at the inner entrance of the internal auditory meatus (meatus acousticus internus). They derive from the nerve sheaths of the upper part of the nervus
662:
Meningiomas and schwannomas occur in around half of patients with NF2. Meningiomas are tumors that are both intracranial and intraspinal. Schwannomas are tumors that are often centered on the internal auditory canal. Patients with NF2 who have meningiomas have a higher risk of mortality, and the
249:
of merlin proteins have been associated with early tumor onset and poor prognosis in people with NF2. Protein truncating mutations correlate with more severe phenotype. There is a broad clinical spectrum known, but all people with the condition who have been checked have been found to have some
646:
Auditory
Brainstem Implants, or ABIs, are used when the cochlea or any portion of the cochleovestibular nerve are not functioning due to damage to those areas or anatomic abnormalities. The procedure is done by implanting a device that send an electrical signal directly to the cochlear nucleus,
603:
is a conservative alternative to cranial base or other intracranial surgery. With conformal radiosurgical techniques, therapeutic radiation focused on the tumour, sparing exposure to surrounding normal tissues. Although radiosurgery can seldom completely destroy a tumor, it can often arrest its
331:
CRL4(DCAF1). Finally, most recent studies indicated that Merlin also plays important role in energy metabolism regulation. Mutations of NF2 is presumed to result in either a failure to synthesize Merlin or the production of a defective peptide that lacks the normal tumor-suppressive effect. The
628:
As hearing loss in individuals with NF2 is generally gradual, eventually profound and sensorineural, the best options for treatment for hearing loss are cochlear implants and auditory brainstem implants (ABIs), as well as supplementing hearing with lip-reading, cued speech or sign language.
663:
treatment can be very challenging. Individuals who develop schwannomas frequently develop hearing loss and deafness. These individuals may also develop tinnitus after being presented with unilateral hearing loss. The first symptom that individuals may encounter is dizziness or imbalance.
559:
The criteria have varied over time. The last revision of the NF2 criteria was done by M.J. Smith in 2017. This included the consideration of a LZTR1 mutation (schwannomatosis) instead of NF2 and excluded bilateral vestibular schwannomas that occur after 70 years of age.
286:, located on chromosome 22 band q11-13.1. Merlin was first discovered as a structural protein functioning as an actin cytoskeleton regulator. Later merlin's tumour suppressant role was described. Merlin regulates multiple proliferative signalling cascades such as
209:, which can sometimes restore a high level of auditory function even when natural hearing is totally lost. However, the amount of destruction to the cochlear nerve caused by the typical NF2 schwannoma often precludes the use of such an implant. In these cases, an
604:
growth or reduce its size. While radiation is less immediately damaging than conventional surgery, it incurs a higher risk of subsequent malignant change in the irradiated tissues, and this risk is higher in NF2 than in sporadic (non-NF2) lesions.
2740:
103:. Besides, other benign brain and spinal tumors occur. Symptoms depend on the presence, localisation and growth of the tumor(s). Many people with this condition also experience vision problems. Neurofibromatosis type II (NF2
593:
used for even small tumors. Small, lateralized tumours in people with NF2 with good hearing should have the middle fossa approach. When the location of the tumour is more medial a retrosigmoid approach may be better.
2593:
2311:
1917:
1893:
573:
There are several different surgical techniques for the removal of acoustic neuroma. The choice of approach is determined by size of the tumour, hearing capability, and general clinical condition of the person.
2252:
587:
Less invasive endoscopic techniques have been done outside of the United States for some time. Recovery times are reported to be faster. However, this technique is not yet mainstream among surgeons in the
165:
Symptoms can occur at any age, typically in adolescence and early adulthood, and rarely seen in children, and the severity depends on the location of the tumours. Symptoms include, but are not limited to:
241:, type of mutations vary and include protein-truncating alterations (frameshift deletions/insertions and nonsense mutations), splice-site mutations, missense mutations and others. Deletions, too, in the
2733:
1128:
Cooper J, Li W, You L, Schiavon G, Pepe-Caprio A, Zhou L, et al. (August 2011). "Merlin/NF2 functions upstream of the nuclear E3 ubiquitin ligase CRL4DCAF1 to suppress oncogenic gene expression".
3525:
2726:
126:
removal of the tumors and surgical treatment of the eye lesions. Historically the underlying disorder has not had any therapy due to the cell function caused by the genetic mutation.
855:
Walter J, Kuhn SA, Brodhun M, Reichart R, Kalff R (June 2009). "Pulmonary meningioma and neurinoma associated with multiple CNS tumours in a patient with neurofibromatosis type 2".
2693:
433:
This would be extremely valuable for the prediction of disease progression and the planning of therapy starting at a young age. The results of such studies are the following:
1618:
Neff BA, Wiet RM, Lasak JM, Cohen NL, Pillsbury HC, Ramsden RT, Welling DB (June 2007). "Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up".
348:. Mutations in the Schwannomin-gene are thought to alter the movement and shape of affected cells with loss of contact inhibition. Ependymomas are tumors arising from the
424:
vestibularis in the region between the central and peripheral myelin (Obersteiner-Redlich-Zone) within the area of the porus acousticus, 1 cm from the brainstem.
581:
The translabyrinthine approach will sacrifice hearing on that side, but will usually spare the facial nerve. Post-operative cerebrospinal fluid leaks are more common.
1520:
3495:
197:
Because hearing loss in those with NF2 almost always occurs after acquisition of verbal language skills, people with NF2 do not always integrate well into
1543:"Bevacizumab in neurofibromatosis type 2 (NF2) related vestibular schwannomas: a nationally coordinated approach to delivery and prospective evaluation"
659:
An early diagnosis is the best way to ensure improvement in management. Although, even with an early diagnosis, some patients still die very young.
905:"Merlin, a "magic" linker between extracellular cues and intracellular signaling pathways that regulate cell motility, proliferation, and survival"
743:
2224:
584:
The middle fossa approach is preferred for small tumors and offers the highest probability of retention of hearing and vestibular function.
356:. In people with NF2 and ependymomas, the tumor suppressant function of Merlin may be compromised. Loss of function mutations occurring in
1081:"The NF2 tumor suppressor gene product, merlin, inhibits cell proliferation and cell cycle progression by repressing cyclin D1 expression"
2556:
149:
form is characterized by multiple cerebral and spinal lesions in people younger than 20 years and with rapid progression of the tumours.
3273:
2125:
612:
There are no prescription medicines currently indicated for reduction in tumor burden for NF2 patients, although in patient studies
3125:
1426:
2871:
2677:
2601:
2525:
2171:
299:
1590:
759:"The neurofibromatosis 2 tumor suppressor gene product, merlin, regulates human meningioma cell growth by signaling through YAP"
3452:
3204:
2042:
303:
1493:
1359:
3480:
2988:
2881:
2191:
2943:
2363:
2217:
1932:
3342:
2812:
2792:
1516:
538:
Unilateral VS plus any two of meningioma, glioma, neurofibroma, schwannoma, posterior subcapsular lenticular opacities
478:
These results suggest that other factors (environment, other mutations) will probably determine the clinical outcome.
3357:
152:
People with NF2 who develop single central tumours with slow progression after the age of 20 are thought to have the
2802:
3052:
2861:
2817:
2551:
1381:
Ferner, Rosalie E., Susan M. Huson, and D. Gareth R. Evans. Neurofibromatoses in clinical practice. Springer, 2011.
667:
exons 9–15. Patients with a missense mutation have a greater survival rate than nonsense and frameshift mutations.
3515:
254:. Through statistics, it is suspected that one-half of cases are inherited, and one-half are the result of new,
2718:
2856:
2755:
2210:
310:. In a normal cell, the concentrations of active (dephosphorylated) merlin are controlled by processes such as
3060:
3008:
2926:
2358:
2072:
3302:
3278:
3256:
3084:
2876:
2530:
2403:
2130:
694:"Neurofibromatosis Type 2 (NF2) and the Implications for Vestibular Schwannoma and Meningioma Pathogenesis"
2705:
Note: Not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see
3099:
3093:
2110:
2099:
3184:
2807:
2186:
2035:
210:
372:
of merlin proteins have been associated with early tumor onset and poor prognosis in affected people.
3142:
3036:
2971:
2764:
2615:
2067:
552:
First degree relative with NF2 and the occurrence of neurofibroma, meningiomas, glioma, or
Schwannoma
474:
Cases are published in which exactly the same mutation is associated with clearly different outcomes.
322:
was described. It is known that Merlin's deficiency can result in unmediated progression through the
1863:
1534:
1419:
67:
3466:
3156:
2866:
2840:
2772:
2454:
2449:
2241:
2150:
1943:
287:
1435:"Revisiting neurofibromatosis type 2 diagnostic criteria to exclude LZTR1-related schwannomatosis"
1220:
Stepanova DS, Semenova G, Kuo YM, Andrews AJ, Ammoun S, Hanemann CO, Chernoff J (September 2017).
314:(which would indicate the need to restrain cell division). It has been shown that Merlin inhibits
3244:
3191:
2997:
2233:
307:
3170:
2848:
2373:
2368:
2302:
2181:
2095:
353:
1983:
1483:
1349:
380:
3520:
3288:
2670:
2629:
2353:
2176:
2028:
1541:
Morris KA, Golding JF, Axon PR, Afridi S, Blesing C, Ferner RE, et al. (December 2016).
1406:
1079:
Xiao GH, Gallagher R, Shetler J, Skele K, Altomare DA, Pestell RG, et al. (March 2005).
737:
555:
First degree relative with NF2 and the occurrence of juvenile posterior subcapsular cataract.
357:
291:
251:
691:
2634:
2444:
1433:
Smith MJ, Bowers NL, Bulman M, Gokhale C, Wallace AJ, King AT, et al. (January 2017).
965:
202:
647:
allowing sound to bypass the peripheral auditory system and straight into the brain stem.
8:
2477:
1921:
1391:
1044:
Sherman LS, Gutmann DH (November 2001). "Merlin: hanging tumor suppression on the Rac".
969:
757:
Striedinger K, VandenBerg SR, Baia GS, McDermott MW, Gutmann DH, Lal A (November 2008).
541:
Two or more meningioma plus unilateral VS or any two of glioma, schwannoma and cataract.
318:
which is crucial for cell motility and tumour invasion. Also, merlin's interaction with
39:
3326:
3196:
2957:
2852:
2782:
2641:
2583:
2090:
1926:
1851:
1796:
1769:
1745:
1718:
1689:
1662:
1643:
1567:
1542:
1459:
1434:
1325:
1300:
1299:
Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (June 2009).
1246:
1221:
1197:
1172:
1153:
1105:
1080:
986:
953:
929:
904:
880:
832:
807:
806:
Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (June 2009).
783:
758:
720:
693:
230:
135:
95:, which is the "auditory-vestibular nerve" that transmits sensory information from the
92:
1661:
Wong K, Kozin ED, Kanumuri VV, Vachicouras N, Miller J, Lacour S, et al. (2019).
1316:
1222:"An Essential Role for the Tumor-Suppressor Merlin in Regulating Fatty Acid Synthesis"
1057:
823:
2907:
2654:
2398:
2263:
2142:
1954:
1832:
1801:
1750:
1694:
1635:
1572:
1489:
1464:
1355:
1330:
1251:
1202:
1145:
1110:
1061:
1026:
991:
934:
872:
837:
788:
725:
455:
448:
52:
1994:
1647:
1096:
884:
225:
3404:
3321:
3218:
3113:
3022:
2706:
2472:
2115:
1791:
1781:
1740:
1730:
1684:
1674:
1627:
1562:
1554:
1454:
1446:
1320:
1312:
1241:
1233:
1192:
1184:
1157:
1137:
1100:
1092:
1053:
1018:
981:
973:
924:
916:
868:
864:
827:
819:
778:
770:
715:
705:
416:
328:
278:
NF2 is caused by a defect in the gene that normally gives rise to a product called
206:
112:
57:
1237:
977:
692:
Bachir S, Shah S, Shapiro S, Koehler A, Mahammedi A, Samy RN; et al. (2021).
3316:
3283:
3210:
2921:
2777:
2748:
2546:
2390:
2120:
1948:
1631:
1450:
2202:
1997:
1384:
1269:
1009:
Okada T, You L, Giancotti FG (May 2007). "Shedding light on Merlin's wizardry".
3438:
3296:
3224:
2515:
2506:
2436:
2105:
920:
578:
The retrosigmoid approach offers some opportunity for the retention of hearing.
468:
361:
119:
2008:
1959:
1906:
1824:
1141:
1022:
237:
NF2 is caused by inactivating mutations in the NF2 gene located at 22q12.2 of
33:
multiple inherited schwannomas, meningiomas, and ependymomas (MISME syndrome))
3509:
2624:
2510:
2426:
1679:
1188:
345:
311:
295:
238:
1902:
616:
has resulted in reduction in tumor growth rates and hearing improvements in
2891:
2661:
2649:
2378:
2321:
2059:
2051:
1836:
1805:
1786:
1754:
1735:
1698:
1639:
1576:
1468:
1334:
1255:
1206:
1149:
1114:
1065:
1030:
995:
938:
876:
841:
792:
729:
600:
532:
486:
341:
198:
123:
3267:
3075:
2520:
2482:
2348:
2316:
2268:
2003:
1978:
1558:
710:
613:
399:
214:
100:
88:
1885:
47:
Figure of various morbidities associated with neurofibromatosis type II.
3385:
2665:
2578:
2421:
524:
516:
444:
420:
388:
384:
365:
333:
323:
242:
1663:"Auditory Brainstem Implants: Recent Progress and Future Perspectives"
1173:"A new concept in NF2 pharmacotherapy: targeting fatty acid synthesis"
774:
3371:
2831:
2340:
2288:
2082:
1989:
437:
In most cases the mutation in the NF2 gene causes shortened peptides.
392:
96:
61:
2570:
2413:
349:
319:
258:
108:
756:
407:
3432:
3424:
3418:
3351:
3136:
1770:"Neurofibromatosis type 2 (NF2): a clinical and molecular review"
1719:"Neurofibromatosis type 2 (NF2): A clinical and molecular review"
337:
1937:
1509:
364:, or the creation of new tumorous cells. Deletions, too, in the
3460:
3446:
3365:
3310:
3071:
3016:
2498:
2331:
2020:
1897:
528:
511:
Ferner et al. give three sets of diagnostic criteria for NF2:
462:
85:
2694:
WHO classification of the tumors of the central nervous system
954:"Molecular insights into NF2/Merlin tumor suppressor function"
3526:
Deficiencies of intracellular signaling peptides and proteins
3178:
3164:
3150:
3107:
3002:
2965:
2951:
2937:
2749:
Deficiencies of intracellular signaling peptides and proteins
1481:
549:
Detection of bilateral acoustic neuroma by imaging-procedures
1660:
3474:
3379:
3238:
3232:
3030:
2982:
2915:
2901:
1910:
1219:
315:
115:
1712:
1710:
1708:
1078:
854:
213:(ABI) can restore some level of hearing, supplemented by
1432:
535:, schwannoma, posterior subcapsular lenticular opacities
503:
Bilateral vestibular schwannomas are diagnostic of NF2.
1540:
1521:
National
Institute of Neurological Disorders and Stroke
1298:
805:
1705:
1613:
1611:
1170:
327:
nucleus and suppresses proliferation by inhibiting E3
641:
545:
Another set of diagnostic criteria is the following:
1875:
1347:
118:, which seems to influence the form and movement of
1608:
1127:
1617:
2232:
1008:
675:Incidence of the condition is about 1 in 60,000.
519:(VS) or family history of NF2 plus Unilateral VS
427:
3507:
340:shows similarities to proteins that connect the
1392:"Neurofibromatosis Type 2: eMedicine Radiology"
951:
360:, where Merlin proteins are coded, can promote
1475:
1171:Stepanova DS, Braun L, Chernoff J (May 2018).
1043:
898:
896:
894:
3496:intracellular signaling peptides and proteins
2734:
2218:
2036:
1354:. Cambridge University Press. pp. 231–.
902:
391:, a tumor seen in neurofibromatosis type II.
1761:
1341:
742:: CS1 maint: multiple names: authors list (
465:, there is no good correlation to determine.
233:fashion, as well as through random mutation.
2557:Embryonal tumour with multilayered rosettes
891:
848:
750:
685:
2741:
2727:
2225:
2211:
2043:
2029:
201:and are more likely to resort to auditory
66:
38:
3358:EDARADD Hypohidrotic ectodermal dysplasia
1795:
1785:
1744:
1734:
1688:
1678:
1566:
1458:
1324:
1245:
1196:
1104:
985:
945:
928:
831:
782:
719:
709:
623:
1482:Jean RĂ©gis; Pierre-Hugues Roche (2008).
1348:Christopher Gillberg (16 October 2003).
485:
406:
398:
379:
264:
224:
2678:Malignant peripheral nerve sheath tumor
2602:Primary central nervous system lymphoma
2526:Dysembryoplastic neuroepithelial tumour
336:. Comparison of Schwannomin with other
134:NF2 is an inheritable disorder with an
3508:
952:Cooper J, Giancotti FG (August 2014).
903:Stamenkovic I, Yu Q (September 2010).
122:. The principal treatments consist of
2722:
2206:
2024:
1831:. University of Washington, Seattle.
1822:
1767:
1716:
1485:Modern Management of Acoustic Neuroma
1294:
1292:
1290:
909:Current Protein & Peptide Science
188:Numbness or weakness in arms and legs
160:
2989:Neutrophil immunodeficiency syndrome
2872:Albright's hereditary osteodystrophy
2192:Encephalocraniocutaneous lipomatosis
1517:"Neurofibromatosis Information Page"
1488:. Karger Publishers. pp. 191–.
1274:The Lecturio Medical Concept Library
632:
332:Schwannomin-peptide consists of 595
3343:Signal transducing adaptor proteins
2364:Subependymal giant cell astrocytoma
857:Clinical Neurology and Neurosurgery
352:, an epithelium-like tissue of the
13:
2927:KRAS Cardiofaciocutaneous syndrome
2818:X-linked intellectual disability 1
2813:Juvenile primary lateral sclerosis
1816:
1287:
642:Auditory Brainstem Implants (ABIs)
440:There are no mutational hot spots.
14:
3537:
3274:Bannayan–Riley–Ruvalcaba syndrome
2126:Bannayan–Riley–Ruvalcaba syndrome
1871:
1774:Orphanet Journal of Rare Diseases
1723:Orphanet Journal of Rare Diseases
411:Meningiomas in a person with NFII
403:Schwannoma of the N. Vestibularis
129:
16:Type of neurofibromatosis disease
2862:Progressive osseous heteroplasia
2552:Atypical teratoid rhabdoid tumor
2050:
141:There are two forms of the NF2:
3467:PRKCSH Polycystic liver disease
2172:Abdallat–Davis–Farrage syndrome
1654:
1583:
1375:
1262:
1213:
1164:
1121:
1097:10.1128/MCB.25.6.2384-2394.2005
461:In cases with mutations in the
273:
192:
3453:Wolff–Parkinson–White syndrome
2857:Pseudopseudohypoparathyroidism
2756:GTP-binding protein regulators
2646:Cranial and paraspinal nerves
1351:Clinical Child Neuropsychiatry
1085:Molecular and Cellular Biology
1072:
1037:
1002:
869:10.1016/j.clineuro.2008.11.018
799:
607:
428:Genotype–phenotype correlation
205:. One of these devices is the
1:
3061:Cardiofaciocutaneous syndrome
3009:Chylomicron retention disease
2359:Pleomorphic xanthoastrocytoma
2234:Tumours of the nervous system
1825:"NF2-Related Schwannomatosis"
1317:10.1016/S0140-6736(09)60259-2
1238:10.1158/0008-5472.CAN-16-2834
1058:10.1016/S0962-8924(01)02128-6
978:10.1016/j.febslet.2014.04.001
824:10.1016/S0140-6736(09)60259-2
678:
670:
270:which lead to the inner ear.
250:mutation of the same gene on
3303:X-linked myotubular myopathy
2404:Anaplastic oligodendroglioma
1632:10.1097/MLG.0b013e31804b1ae7
1451:10.1212/WNL.0000000000003418
650:
563:
506:
481:
471:may have only minor effects.
375:
229:NF-2 may be inherited in an
7:
3100:X-linked agammaglobulinemia
2944:Charcot–Marie–Tooth disease
2111:Multiple hamartoma syndrome
498:
91:in the nerve sheath of the
10:
3542:
2803:Marinesco–Sjögren syndrome
1717:Evans, Dgareth R. (2009).
1591:"Cochlear Implant Surgery"
1301:"Neurofibromatosis type 2"
921:10.2174/138920310791824011
808:"Neurofibromatosis type 2"
568:
211:auditory brainstem implant
3491:
3411:Neurofibromatosis type II
3396:
3341:
3255:
3245:Pseudohypoaldosteronism 2
3124:
3083:
3069:
3051:
2972:Griscelli syndrome type 2
2890:
2839:
2830:
2791:
2765:GTPase-activating protein
2763:
2754:
2702:
2686:
2614:
2592:
2569:
2539:
2495:
2465:
2435:
2412:
2389:
2339:
2330:
2310:
2301:
2277:
2249:
2240:
2164:
2141:
2081:
2073:Von Hippel–Lindau disease
2058:
1969:
1879:
1823:Evans, D. Gareth (1993).
1667:Frontiers in Neuroscience
1142:10.1126/scisignal.2002314
1023:10.1016/j.tcb.2007.03.006
154:Feiling–Gardner phenotype
78:Neurofibromatosis type II
51:
46:
37:
29:
25:Neurofibromatosis type II
24:
3279:Lhermitte–Duclos disease
2877:McCune–Albright syndrome
2867:Pseudohypoparathyroidism
2773:Neurofibromatosis type I
2531:Lhermitte–Duclos disease
2455:Choroid plexus carcinoma
2450:Choroid plexus papilloma
2131:Lhermitte–Duclos disease
1680:10.3389/fnins.2019.00010
1189:10.18632/oncoscience.417
458:have a better prognosis.
288:receptor tyrosine kinase
220:
173:Loss/problems of balance
3037:Bardet–Biedl syndrome 3
2100:Pallister–Hall syndrome
1595:www.hopkinsmedicine.org
1547:Neuro-Oncology Practice
3516:Neurological disorders
3185:Peutz–Jeghers syndrome
3171:Incontinentia pigmenti
3157:Li–Fraumeni syndrome 2
2808:Aarskog–Scott syndrome
2374:Anaplastic astrocytoma
2369:Fibrillary astrocytoma
2187:Peutz–Jeghers syndrome
2182:Incontinentia pigmenti
2096:Hypothalamic hamartoma
1787:10.1186/1750-1172-4-16
1768:Evans DG (June 2009).
1736:10.1186/1750-1172-4-16
1414:Cite journal requires
1046:Trends in Cell Biology
1011:Trends in Cell Biology
624:Treatment hearing loss
491:
463:splice-acceptor-region
412:
404:
396:
354:central nervous system
234:
138:mode of transmission.
3289:Proteus-like syndrome
3143:Coffin-Lowry syndrome
2630:Esthesioneuroblastoma
2354:Pilocytic astrocytoma
2177:Ataxia telangiectasia
2068:Sturge–Weber syndrome
515:Bilateral vestibular
489:
410:
402:
383:
265:Cause loss of hearing
228:
3197:Myotonic dystrophy 1
2635:Ganglioneuroblastoma
2540:CNS embryonal tumors
2445:Choroid plexus tumor
711:10.3390/ijms22020690
451:have poor prognosis.
445:frameshift mutations
419:of NF2 is in fact a
292:p21-activated kinase
203:assistive technology
107:NF II) is caused by
2478:Gliomatosis cerebri
2009:Neurofibromatosis 2
1829:Neurofibromatosis 2
1311:(9679): 1974–1986.
970:2014FEBSL.588.2743C
818:(9679): 1974–1986.
3327:Metachondromatosis
3023:Joubert syndrome 8
2958:Carpenter syndrome
2793:Guanine nucleotide
2783:Tuberous sclerosis
2642:Nerve sheath tumor
2584:Hemangiopericytoma
2091:Tuberous sclerosis
1970:External resources
1559:10.1093/nop/npv065
492:
456:missense mutations
449:nonsense mutations
413:
405:
397:
235:
231:autosomal dominant
161:Symptoms and signs
136:autosomal dominant
93:cranial nerve VIII
3503:
3502:
3337:
3336:
3317:Noonan syndrome 1
3211:Seckel syndrome 1
3047:
3046:
2922:Noonan syndrome 3
2908:Costello syndrome
2826:
2825:
2716:
2715:
2655:Neurofibromatosis
2610:
2609:
2565:
2564:
2491:
2490:
2399:Oligodendroglioma
2297:
2296:
2264:Craniopharyngioma
2200:
2199:
2143:Neurofibromatosis
2018:
2017:
1495:978-3-8055-8370-1
1361:978-0-521-54335-4
1232:(18): 5026–5038.
1130:Science Signaling
964:(16): 2743–2752.
775:10.1593/neo.08642
769:(11): 1204–1212.
633:Cochlear Implants
185:Vision impairment
147:Wishart-phenotype
75:
74:
19:Medical condition
3533:
3322:LEOPARD syndrome
3225:Oguchi disease 2
3126:Serine/threonine
3114:ZAP70 deficiency
3081:
3080:
2837:
2836:
2761:
2760:
2743:
2736:
2729:
2720:
2719:
2707:brain metastasis
2671:Acoustic neuroma
2473:Oligoastrocytoma
2466:Multiple/unknown
2337:
2336:
2328:
2327:
2308:
2307:
2282:
2257:
2247:
2246:
2227:
2220:
2213:
2204:
2203:
2116:Proteus syndrome
2045:
2038:
2031:
2022:
2021:
1877:
1876:
1867:
1861:
1857:
1855:
1847:
1845:
1843:
1810:
1809:
1799:
1789:
1765:
1759:
1758:
1748:
1738:
1714:
1703:
1702:
1692:
1682:
1658:
1652:
1651:
1626:(6): 1069–1072.
1620:The Laryngoscope
1615:
1606:
1605:
1603:
1602:
1587:
1581:
1580:
1570:
1538:
1532:
1531:
1529:
1527:
1513:
1507:
1506:
1504:
1502:
1479:
1473:
1472:
1462:
1430:
1424:
1423:
1417:
1412:
1410:
1402:
1400:
1399:
1388:
1382:
1379:
1373:
1372:
1370:
1368:
1345:
1339:
1338:
1328:
1296:
1285:
1284:
1282:
1280:
1266:
1260:
1259:
1249:
1217:
1211:
1210:
1200:
1183:(5–6): 126–127.
1168:
1162:
1161:
1125:
1119:
1118:
1108:
1091:(6): 2384–2394.
1076:
1070:
1069:
1041:
1035:
1034:
1006:
1000:
999:
989:
949:
943:
942:
932:
900:
889:
888:
852:
846:
845:
835:
803:
797:
796:
786:
754:
748:
747:
741:
733:
723:
713:
689:
417:acoustic neuroma
370:-terminal domain
329:ubiquitin ligase
247:-terminal domain
207:cochlear implant
71:
70:
58:Medical genetics
42:
22:
21:
3541:
3540:
3536:
3535:
3534:
3532:
3531:
3530:
3506:
3505:
3504:
3499:
3487:
3392:
3333:
3284:Cowden syndrome
3258:
3251:
3127:
3120:
3085:Tyrosine kinase
3065:
3043:
2886:
2822:
2795:exchange factor
2794:
2787:
2778:Watson syndrome
2750:
2747:
2717:
2712:
2711:
2698:
2682:
2606:
2588:
2561:
2547:Medulloblastoma
2535:
2497:
2487:
2461:
2431:
2408:
2391:Oligodendrocyte
2385:
2320:
2314:
2312:Neuroepithelial
2293:
2278:
2273:
2250:
2236:
2231:
2201:
2196:
2160:
2137:
2121:Cowden syndrome
2077:
2054:
2049:
2019:
2014:
2013:
1965:
1964:
1888:
1874:
1859:
1858:
1849:
1848:
1841:
1839:
1819:
1817:Further reading
1814:
1813:
1766:
1762:
1715:
1706:
1659:
1655:
1616:
1609:
1600:
1598:
1597:. 8 August 2021
1589:
1588:
1584:
1539:
1535:
1525:
1523:
1515:
1514:
1510:
1500:
1498:
1496:
1480:
1476:
1431:
1427:
1415:
1413:
1404:
1403:
1397:
1395:
1390:
1389:
1385:
1380:
1376:
1366:
1364:
1362:
1346:
1342:
1297:
1288:
1278:
1276:
1268:
1267:
1263:
1226:Cancer Research
1218:
1214:
1169:
1165:
1126:
1122:
1077:
1073:
1052:(11): 442–444.
1042:
1038:
1007:
1003:
950:
946:
901:
892:
853:
849:
804:
800:
755:
751:
735:
734:
690:
686:
681:
673:
653:
644:
635:
626:
610:
571:
566:
509:
501:
484:
469:Point mutations
430:
378:
369:
308:MST-YAP cascade
276:
267:
246:
223:
195:
163:
132:
80:(also known as
65:
20:
17:
12:
11:
5:
3539:
3529:
3528:
3523:
3518:
3501:
3500:
3492:
3489:
3488:
3486:
3485:
3484:
3483:
3471:
3470:
3469:
3457:
3456:
3455:
3443:
3442:
3441:
3439:Carney complex
3429:
3428:
3427:
3415:
3414:
3413:
3400:
3398:
3394:
3393:
3391:
3390:
3389:
3388:
3376:
3375:
3374:
3362:
3361:
3360:
3347:
3345:
3339:
3338:
3335:
3334:
3332:
3331:
3330:
3329:
3324:
3319:
3307:
3306:
3305:
3293:
3292:
3291:
3286:
3281:
3276:
3263:
3261:
3253:
3252:
3250:
3249:
3248:
3247:
3229:
3228:
3227:
3215:
3214:
3213:
3201:
3200:
3199:
3189:
3188:
3187:
3175:
3174:
3173:
3161:
3160:
3159:
3147:
3146:
3145:
3132:
3130:
3122:
3121:
3119:
3118:
3117:
3116:
3104:
3103:
3102:
3089:
3087:
3078:
3067:
3066:
3064:
3063:
3057:
3055:
3049:
3048:
3045:
3044:
3042:
3041:
3040:
3039:
3027:
3026:
3025:
3013:
3012:
3011:
2994:
2993:
2992:
2991:
2977:
2976:
2975:
2974:
2962:
2961:
2960:
2948:
2947:
2946:
2932:
2931:
2930:
2929:
2924:
2912:
2911:
2910:
2896:
2894:
2888:
2887:
2885:
2884:
2879:
2874:
2869:
2864:
2859:
2845:
2843:
2834:
2828:
2827:
2824:
2823:
2821:
2820:
2815:
2810:
2805:
2799:
2797:
2789:
2788:
2786:
2785:
2780:
2775:
2769:
2767:
2758:
2752:
2751:
2746:
2745:
2738:
2731:
2723:
2714:
2713:
2703:
2700:
2699:
2697:
2696:
2690:
2688:
2684:
2683:
2681:
2680:
2675:
2674:
2673:
2659:
2658:
2657:
2652:
2644:
2639:
2638:
2637:
2632:
2621:
2619:
2612:
2611:
2608:
2607:
2605:
2604:
2598:
2596:
2590:
2589:
2587:
2586:
2581:
2575:
2573:
2567:
2566:
2563:
2562:
2560:
2559:
2554:
2549:
2543:
2541:
2537:
2536:
2534:
2533:
2528:
2523:
2518:
2516:Retinoblastoma
2513:
2507:Ganglioneuroma
2503:
2501:
2493:
2492:
2489:
2488:
2486:
2485:
2480:
2475:
2469:
2467:
2463:
2462:
2460:
2459:
2458:
2457:
2452:
2441:
2439:
2437:Choroid plexus
2433:
2432:
2430:
2429:
2424:
2418:
2416:
2410:
2409:
2407:
2406:
2401:
2395:
2393:
2387:
2386:
2384:
2383:
2382:
2381:
2376:
2371:
2366:
2361:
2356:
2345:
2343:
2334:
2325:
2305:
2299:
2298:
2295:
2294:
2292:
2291:
2285:
2283:
2275:
2274:
2272:
2271:
2266:
2260:
2258:
2244:
2238:
2237:
2230:
2229:
2222:
2215:
2207:
2198:
2197:
2195:
2194:
2189:
2184:
2179:
2174:
2168:
2166:
2162:
2161:
2159:
2158:
2153:
2147:
2145:
2139:
2138:
2136:
2135:
2134:
2133:
2128:
2123:
2118:
2108:
2106:Megalencephaly
2103:
2093:
2087:
2085:
2079:
2078:
2076:
2075:
2070:
2064:
2062:
2056:
2055:
2048:
2047:
2040:
2033:
2025:
2016:
2015:
2012:
2011:
2000:
1986:
1974:
1973:
1971:
1967:
1966:
1963:
1962:
1951:
1940:
1929:
1914:
1889:
1884:
1883:
1881:
1880:Classification
1873:
1872:External links
1870:
1869:
1868:
1860:|journal=
1818:
1815:
1812:
1811:
1760:
1704:
1653:
1607:
1582:
1553:(4): 281–289.
1533:
1508:
1494:
1474:
1425:
1416:|journal=
1383:
1374:
1360:
1340:
1286:
1261:
1212:
1163:
1120:
1071:
1036:
1017:(5): 222–229.
1001:
944:
915:(6): 471–484.
890:
863:(5): 454–459.
847:
798:
749:
683:
682:
680:
677:
672:
669:
652:
649:
643:
640:
634:
631:
625:
622:
609:
606:
590:
589:
585:
582:
579:
570:
567:
565:
562:
557:
556:
553:
550:
543:
542:
539:
536:
508:
505:
500:
497:
483:
480:
476:
475:
472:
466:
459:
452:
441:
438:
429:
426:
415:The so-called
377:
374:
367:
358:chromosome 22q
275:
272:
266:
263:
244:
222:
219:
194:
191:
190:
189:
186:
183:
180:
177:
174:
171:
162:
159:
158:
157:
150:
131:
130:Classification
128:
82:MISME syndrome
73:
72:
55:
49:
48:
44:
43:
35:
34:
31:
27:
26:
18:
15:
9:
6:
4:
3:
2:
3538:
3527:
3524:
3522:
3519:
3517:
3514:
3513:
3511:
3498:
3497:
3490:
3482:
3479:
3478:
3477:
3476:
3472:
3468:
3465:
3464:
3463:
3462:
3458:
3454:
3451:
3450:
3449:
3448:
3444:
3440:
3437:
3436:
3435:
3434:
3430:
3426:
3423:
3422:
3421:
3420:
3416:
3412:
3409:
3408:
3407:
3406:
3402:
3401:
3399:
3395:
3387:
3384:
3383:
3382:
3381:
3377:
3373:
3370:
3369:
3368:
3367:
3363:
3359:
3356:
3355:
3354:
3353:
3349:
3348:
3346:
3344:
3340:
3328:
3325:
3323:
3320:
3318:
3315:
3314:
3313:
3312:
3308:
3304:
3301:
3300:
3299:
3298:
3294:
3290:
3287:
3285:
3282:
3280:
3277:
3275:
3272:
3271:
3270:
3269:
3265:
3264:
3262:
3260:
3254:
3246:
3243:
3242:
3241:
3240:
3235:
3234:
3230:
3226:
3223:
3222:
3221:
3220:
3216:
3212:
3209:
3208:
3207:
3206:
3202:
3198:
3195:
3194:
3193:
3190:
3186:
3183:
3182:
3181:
3180:
3176:
3172:
3169:
3168:
3167:
3166:
3162:
3158:
3155:
3154:
3153:
3152:
3148:
3144:
3141:
3140:
3139:
3138:
3134:
3133:
3131:
3129:
3123:
3115:
3112:
3111:
3110:
3109:
3105:
3101:
3098:
3097:
3096:
3095:
3091:
3090:
3088:
3086:
3082:
3079:
3077:
3073:
3068:
3062:
3059:
3058:
3056:
3054:
3050:
3038:
3035:
3034:
3033:
3032:
3028:
3024:
3021:
3020:
3019:
3018:
3014:
3010:
3007:
3006:
3005:
3004:
2999:
2996:
2995:
2990:
2987:
2986:
2985:
2984:
2979:
2978:
2973:
2970:
2969:
2968:
2967:
2963:
2959:
2956:
2955:
2954:
2953:
2949:
2945:
2942:
2941:
2940:
2939:
2934:
2933:
2928:
2925:
2923:
2920:
2919:
2918:
2917:
2913:
2909:
2906:
2905:
2904:
2903:
2898:
2897:
2895:
2893:
2889:
2883:
2880:
2878:
2875:
2873:
2870:
2868:
2865:
2863:
2860:
2858:
2854:
2850:
2847:
2846:
2844:
2842:
2841:Heterotrimeic
2838:
2835:
2833:
2829:
2819:
2816:
2814:
2811:
2809:
2806:
2804:
2801:
2800:
2798:
2796:
2790:
2784:
2781:
2779:
2776:
2774:
2771:
2770:
2768:
2766:
2762:
2759:
2757:
2753:
2744:
2739:
2737:
2732:
2730:
2725:
2724:
2721:
2710:
2708:
2701:
2695:
2692:
2691:
2689:
2685:
2679:
2676:
2672:
2669:
2668:
2667:
2663:
2660:
2656:
2653:
2651:
2648:
2647:
2645:
2643:
2640:
2636:
2633:
2631:
2628:
2627:
2626:
2625:Neuroblastoma
2623:
2622:
2620:
2617:
2613:
2603:
2600:
2599:
2597:
2595:
2594:Hematopoietic
2591:
2585:
2582:
2580:
2577:
2576:
2574:
2572:
2568:
2558:
2555:
2553:
2550:
2548:
2545:
2544:
2542:
2538:
2532:
2529:
2527:
2524:
2522:
2519:
2517:
2514:
2512:
2511:Ganglioglioma
2508:
2505:
2504:
2502:
2500:
2494:
2484:
2481:
2479:
2476:
2474:
2471:
2470:
2468:
2464:
2456:
2453:
2451:
2448:
2447:
2446:
2443:
2442:
2440:
2438:
2434:
2428:
2427:Subependymoma
2425:
2423:
2420:
2419:
2417:
2415:
2411:
2405:
2402:
2400:
2397:
2396:
2394:
2392:
2388:
2380:
2377:
2375:
2372:
2370:
2367:
2365:
2362:
2360:
2357:
2355:
2352:
2351:
2350:
2347:
2346:
2344:
2342:
2338:
2335:
2333:
2329:
2326:
2323:
2322:spinal tumors
2318:
2313:
2309:
2306:
2304:
2300:
2290:
2287:
2286:
2284:
2281:
2276:
2270:
2267:
2265:
2262:
2261:
2259:
2256:
2254:
2248:
2245:
2243:
2239:
2235:
2228:
2223:
2221:
2216:
2214:
2209:
2208:
2205:
2193:
2190:
2188:
2185:
2183:
2180:
2178:
2175:
2173:
2170:
2169:
2167:
2163:
2157:
2154:
2152:
2149:
2148:
2146:
2144:
2140:
2132:
2129:
2127:
2124:
2122:
2119:
2117:
2114:
2113:
2112:
2109:
2107:
2104:
2101:
2097:
2094:
2092:
2089:
2088:
2086:
2084:
2080:
2074:
2071:
2069:
2066:
2065:
2063:
2061:
2057:
2053:
2046:
2041:
2039:
2034:
2032:
2027:
2026:
2023:
2010:
2006:
2005:
2001:
1999:
1996:
1992:
1991:
1987:
1985:
1981:
1980:
1976:
1975:
1972:
1968:
1961:
1957:
1956:
1952:
1950:
1946:
1945:
1941:
1939:
1935:
1934:
1930:
1928:
1924:
1923:
1919:
1915:
1912:
1908:
1904:
1900:
1899:
1895:
1891:
1890:
1887:
1882:
1878:
1865:
1853:
1838:
1834:
1830:
1826:
1821:
1820:
1807:
1803:
1798:
1793:
1788:
1783:
1779:
1775:
1771:
1764:
1756:
1752:
1747:
1742:
1737:
1732:
1728:
1724:
1720:
1713:
1711:
1709:
1700:
1696:
1691:
1686:
1681:
1676:
1672:
1668:
1664:
1657:
1649:
1645:
1641:
1637:
1633:
1629:
1625:
1621:
1614:
1612:
1596:
1592:
1586:
1578:
1574:
1569:
1564:
1560:
1556:
1552:
1548:
1544:
1537:
1522:
1518:
1512:
1497:
1491:
1487:
1486:
1478:
1470:
1466:
1461:
1456:
1452:
1448:
1444:
1440:
1436:
1429:
1421:
1408:
1393:
1387:
1378:
1363:
1357:
1353:
1352:
1344:
1336:
1332:
1327:
1322:
1318:
1314:
1310:
1306:
1302:
1295:
1293:
1291:
1275:
1271:
1265:
1257:
1253:
1248:
1243:
1239:
1235:
1231:
1227:
1223:
1216:
1208:
1204:
1199:
1194:
1190:
1186:
1182:
1178:
1174:
1167:
1159:
1155:
1151:
1147:
1143:
1139:
1135:
1131:
1124:
1116:
1112:
1107:
1102:
1098:
1094:
1090:
1086:
1082:
1075:
1067:
1063:
1059:
1055:
1051:
1047:
1040:
1032:
1028:
1024:
1020:
1016:
1012:
1005:
997:
993:
988:
983:
979:
975:
971:
967:
963:
959:
955:
948:
940:
936:
931:
926:
922:
918:
914:
910:
906:
899:
897:
895:
886:
882:
878:
874:
870:
866:
862:
858:
851:
843:
839:
834:
829:
825:
821:
817:
813:
809:
802:
794:
790:
785:
780:
776:
772:
768:
764:
760:
753:
745:
739:
731:
727:
722:
717:
712:
707:
703:
699:
698:Int J Mol Sci
695:
688:
684:
676:
668:
664:
660:
657:
656:in mutation.
648:
639:
630:
621:
619:
615:
605:
602:
598:
594:
586:
583:
580:
577:
576:
575:
561:
554:
551:
548:
547:
546:
540:
537:
534:
530:
526:
522:
518:
514:
513:
512:
504:
496:
488:
479:
473:
470:
467:
464:
460:
457:
453:
450:
446:
442:
439:
436:
435:
434:
425:
422:
418:
409:
401:
394:
390:
386:
382:
373:
371:
363:
362:tumorigenesis
359:
355:
351:
347:
346:cell membrane
343:
339:
335:
330:
325:
321:
317:
313:
312:cell adhesion
309:
305:
301:
297:
293:
289:
285:
281:
271:
262:
260:
257:
253:
252:chromosome 22
248:
240:
239:chromosome 22
232:
227:
218:
216:
212:
208:
204:
200:
187:
184:
181:
178:
175:
172:
169:
168:
167:
155:
151:
148:
144:
143:
142:
139:
137:
127:
125:
124:neurosurgical
121:
117:
114:
110:
106:
102:
98:
94:
90:
87:
83:
79:
69:
63:
59:
56:
54:
50:
45:
41:
36:
32:
28:
23:
3521:Neurosurgery
3493:
3473:
3459:
3445:
3431:
3417:
3410:
3403:
3378:
3364:
3350:
3309:
3295:
3266:
3237:
3231:
3217:
3203:
3177:
3163:
3149:
3135:
3106:
3092:
3029:
3015:
3001:
2981:
2964:
2950:
2936:
2914:
2900:
2704:
2662:Neurilemmoma
2650:Neurofibroma
2379:Glioblastoma
2317:brain tumors
2279:
2251:
2155:
2060:Angiomatosis
2052:Phakomatosis
2002:
1988:
1977:
1953:
1942:
1931:
1916:
1892:
1840:. Retrieved
1828:
1777:
1773:
1763:
1726:
1722:
1670:
1666:
1656:
1623:
1619:
1599:. Retrieved
1594:
1585:
1550:
1546:
1536:
1524:. Retrieved
1511:
1499:. Retrieved
1484:
1477:
1445:(1): 87–92.
1442:
1438:
1428:
1407:cite journal
1396:. Retrieved
1394:. 2016-09-26
1386:
1377:
1365:. Retrieved
1350:
1343:
1308:
1304:
1277:. Retrieved
1273:
1270:"Ependymoma"
1264:
1229:
1225:
1215:
1180:
1176:
1166:
1136:(188): pt6.
1133:
1129:
1123:
1088:
1084:
1074:
1049:
1045:
1039:
1014:
1010:
1004:
961:
958:FEBS Letters
957:
947:
912:
908:
860:
856:
850:
815:
811:
801:
766:
762:
752:
738:cite journal
701:
697:
687:
674:
665:
661:
658:
654:
645:
636:
627:
617:
611:
601:Radiosurgery
599:
595:
591:
572:
558:
544:
533:neurofibroma
523:any two of:
520:
510:
502:
493:
477:
454:People with
443:People with
431:
414:
342:cytoskeleton
298:signalling,
294:signalling,
290:signalling,
283:
279:
277:
274:Pathogenesis
268:
255:
236:
199:Deaf culture
196:
193:Hearing loss
182:Hearing loss
164:
153:
146:
140:
133:
104:
89:brain tumors
81:
77:
76:
3259:phosphatase
3076:phosphatase
2521:Neurocytoma
2483:Gliosarcoma
2349:Astrocytoma
2269:Pituicytoma
2004:GeneReviews
1979:MedlinePlus
1501:20 December
1367:20 December
1177:Oncoscience
614:Bevacizumab
608:Medications
334:amino acids
284:Schwannomin
215:lip reading
30:Other names
3510:Categories
3386:Zaspopathy
3053:MAP kinase
2666:Schwannoma
2579:Meningioma
2422:Ependymoma
1955:DiseasesDB
1601:2021-11-29
1398:2010-12-20
704:(2): 690.
679:References
671:Prevalence
620:patients.
525:meningioma
517:schwannoma
490:NF-2 Locus
421:schwannoma
389:schwannoma
385:Micrograph
324:cell cycle
3494:See also
3372:Cherubism
2892:Monomeric
2832:G protein
2341:Astrocyte
2289:Pinealoma
2242:Endocrine
2083:Hamartoma
1998:radio/475
1995:neuro/496
1990:eMedicine
1862:ignored (
1852:cite book
1439:Neurology
763:Neoplasia
651:Prognosis
564:Treatment
507:Postnatal
482:Diagnosis
393:HPS stain
376:Pathology
306:cascade,
259:mutations
109:mutations
97:inner ear
62:neurology
53:Specialty
3257:Tyrosine
2571:Meninges
2414:Ependyma
1913:Q85.020)
1837:20301380
1806:19545378
1755:19545378
1699:30760974
1648:23086289
1640:17545869
1577:29692918
1526:June 16,
1469:27856782
1335:19476995
1256:28729415
1207:30035161
1150:21878678
1115:15743831
1066:11684412
1031:17442573
996:24726726
939:20491622
885:22696343
877:19249154
842:19476995
793:18953429
730:33445724
499:Prenatal
350:ependyma
338:proteins
320:cyclin D
179:Seizures
176:Glaucoma
170:Tinnitus
113:"Merlin"
3433:PRKAR1A
3425:CADASIL
3419:Notch 3
3352:EDARADD
3137:RPS6KA3
2156:Type II
1949:D016518
1797:2708144
1746:2708144
1690:6361749
1568:5909937
1460:5200853
1326:4748851
1279:19 July
1247:5600854
1198:6049319
1158:9582719
1106:1061616
987:4111995
966:Bibcode
930:2946555
833:4748851
784:2570596
721:7828193
569:Surgery
344:to the
256:de novo
111:of the
99:to the
3461:PRKCSH
3447:PRKAG2
3366:SH3BP2
3311:PTPN11
3128:kinase
3072:kinase
3070:Other
3017:ARL13B
2499:neuron
2496:Mature
2332:Glioma
2280:Other:
2253:Sellar
2151:Type I
1984:000795
1938:101000
1927:237.72
1842:30 May
1835:
1804:
1794:
1780:: 16.
1753:
1743:
1729:: 16.
1697:
1687:
1673:: 10.
1646:
1638:
1575:
1565:
1492:
1467:
1457:
1358:
1333:
1323:
1305:Lancet
1254:
1244:
1205:
1195:
1156:
1148:
1113:
1103:
1064:
1029:
994:
984:
937:
927:
883:
875:
840:
830:
812:Lancet
791:
781:
728:
718:
529:glioma
280:Merlin
86:benign
64:
3481:XIAP2
3397:Other
3179:STK11
3165:IKBKG
3151:CHEK2
3108:ZAP70
3003:SAR1B
2980:RHO:
2966:RAB27
2952:RAB23
2935:RAB:
2899:RAS:
2882:CGL 2
2853:GNAS1
2687:Other
2165:Other
1907:Q85.0
1644:S2CID
1154:S2CID
881:S2CID
387:of a
221:Cause
120:cells
101:brain
3475:XIAP
3380:LDB3
3297:MTM1
3268:PTEN
3239:WNK1
3233:WNK4
3219:GRK1
3192:DMPK
3031:ARL6
2983:RAC2
2938:RAB7
2916:KRAS
2902:HRAS
2849:cAMP
1960:8960
1944:MeSH
1933:OMIM
1922:9-CM
1911:ILDS
1864:help
1844:2017
1833:PMID
1802:PMID
1751:PMID
1695:PMID
1636:PMID
1573:PMID
1528:2019
1503:2010
1490:ISBN
1465:PMID
1420:help
1369:2010
1356:ISBN
1331:PMID
1281:2021
1252:PMID
1203:PMID
1146:PMID
1111:PMID
1062:PMID
1027:PMID
992:PMID
935:PMID
873:PMID
838:PMID
789:PMID
744:link
726:PMID
618:some
316:Rac1
145:The
116:gene
3405:NF2
3205:ATR
3094:BTK
2998:ARF
2616:PNS
2303:CNS
1918:ICD
1903:D33
1894:ICD
1792:PMC
1782:doi
1741:PMC
1731:doi
1685:PMC
1675:doi
1628:doi
1624:117
1563:PMC
1555:doi
1455:PMC
1447:doi
1321:PMC
1313:doi
1309:373
1242:PMC
1234:doi
1193:PMC
1185:doi
1138:doi
1101:PMC
1093:doi
1054:doi
1019:doi
982:PMC
974:doi
962:588
925:PMC
917:doi
865:doi
861:111
828:PMC
820:doi
816:373
779:PMC
771:doi
716:PMC
706:doi
588:US.
447:or
304:ERK
300:MEK
296:Ras
282:or
3512::
3000::
2855::
2709:).
2509::
2007::
1993::
1982::
1958::
1947::
1936::
1925::
1905:,
1901::
1898:10
1856::
1854:}}
1850:{{
1827:.
1800:.
1790:.
1776:.
1772:.
1749:.
1739:.
1725:.
1721:.
1707:^
1693:.
1683:.
1671:13
1669:.
1665:.
1642:.
1634:.
1622:.
1610:^
1593:.
1571:.
1561:.
1549:.
1545:.
1519:.
1463:.
1453:.
1443:88
1441:.
1437:.
1411::
1409:}}
1405:{{
1329:.
1319:.
1307:.
1303:.
1289:^
1272:.
1250:.
1240:.
1230:77
1228:.
1224:.
1201:.
1191:.
1179:.
1175:.
1152:.
1144:.
1132:.
1109:.
1099:.
1089:25
1087:.
1083:.
1060:.
1050:11
1048:.
1025:.
1015:17
1013:.
990:.
980:.
972:.
960:.
956:.
933:.
923:.
913:11
911:.
907:.
893:^
879:.
871:.
859:.
836:.
826:.
814:.
810:.
787:.
777:.
767:10
765:.
761:.
740:}}
736:{{
724:.
714:.
702:22
700:.
696:.
531:,
527:,
521:or
366:NH
261:.
243:NH
217:.
105:or
60:,
3236:/
3074:/
2851:/
2742:e
2735:t
2728:v
2664:/
2618::
2324:)
2319:,
2315:(
2255::
2226:e
2219:t
2212:v
2102:)
2098:(
2044:e
2037:t
2030:v
1920:-
1909:(
1896:-
1886:D
1866:)
1846:.
1808:.
1784::
1778:4
1757:.
1733::
1727:4
1701:.
1677::
1650:.
1630::
1604:.
1579:.
1557::
1551:3
1530:.
1505:.
1471:.
1449::
1422:)
1418:(
1401:.
1371:.
1337:.
1315::
1283:.
1258:.
1236::
1209:.
1187::
1181:5
1160:.
1140::
1134:4
1117:.
1095::
1068:.
1056::
1033:.
1021::
998:.
976::
968::
941:.
919::
887:.
867::
844:.
822::
795:.
773::
746:)
732:.
708::
395:.
368:2
302:-
245:2
156:.
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
