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352:(PBG synthase) which increases ALA and precipitates acute neuropathic symptoms, similar to porphyria.
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de Laet C, Dionisi-Vici C, Leonard JV, McKiernan P, Mitchell G, Monti L, et al. (January 2013).
340:. This enzyme is involved in the catabolism of tyrosine, and if deficient, leads to accumulation of
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which is subsequently converted to succinylacetone which can be detected in the urine by
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Except where otherwise noted, data are given for materials in their
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InChI=1S/C7H10O4/c1-5(8)4-6(9)2-3-7(10)11/h2-4H2,1H3,(H,10,11)
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371:"Recommendations for the management of tyrosinaemia type 1"
336:, which is due to congenital deficiency of an enzyme,
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220:Key: WYEPBHZLDUPIOD-UHFFFAOYSA-N
324:that is formed by the oxidation of
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375:Orphanet Journal of Rare Diseases
348:. Succinylacetone also inhibits
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302:(at 25 °C , 100 kPa).
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338:fumarylacetoacetate hydrolase
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41:4,6-Dioxoheptanoic acid
328:and is a precursor of
388:10.1186/1750-1172-8-8
241:CC(=O)CC(=O)CCC(=O)O
37:Preferred IUPAC name
342:fumarylacetoacetate
292: g·mol
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334:tyrosinemia type 1
306:Infobox references
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322:chemical compound
314:Chemical compound
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173:CompTox Dashboard
97:Interactive image
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432:Carboxylic acids
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257:Chemical formula
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50:Succinyl acetone
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17:Succinylacetone
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350:ALA dehydratase
318:Succinylacetone
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190:DTXSID50199519
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56:Identifiers
48:Other names
250:Properties
115:100.153.292
421:Categories
356:References
285:Molar mass
161:KZ0KV2Q190
84:3D model (
73:51568-18-4
63:CAS Number
427:Diketones
407:23311542
398:3558375
326:glycine
290:158.153
128:PubChem
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234:SMILES
31:Names
381:: 8.
320:is a
209:InChI
86:JSmol
403:PMID
346:GCMS
152:UNII
141:5312
393:PMC
383:doi
178:EPA
131:CID
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275:O
269:H
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263:C
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