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Details on Person This is the final step of tyrosine degradation. Fumarylaceto...
| Class:Id | Summation:178797 |
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| _displayName | This is the final step of tyrosine degradation. Fumarylaceto... |
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| _timestamp | 2023-12-08 09:37:09 |
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| created | [InstanceEdit:179407] Gopinathrao, G, 2006-04-27 13:13:48 |
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| modified | [InstanceEdit:517440] D'Eustachio, P, 2010-02-18
[InstanceEdit:8986503] Naithani, Sushma, 2017-04-27
[InstanceEdit:9644829] D'Eustachio, Peter, 2019-04-24
[InstanceEdit:9855935] Stephan, Ralf, 2023-12-08 |
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| text | This is the final step of tyrosine degradation. Fumarylacetoacetase catalyzes the hydrolysis of 4-fumarylacetoacetate (4FAA) to form fumarate and acetoacetate. The enzyme is a homodimer, with each monomer binding one Ca(2+) and one Mg(2+) ion as catalytic cofactors, as shown in a mouse structure (Hsiang et al., 1972; Labelle et al. 1993; Bateman et al., 2001). FAH deficiency leads to tyrosinemia type I (TYRSN1, MIM:276700) characterized by 4FAA accumulation with severe metabolic effects (reviewed in Pitkänen et al., 2000; Scott, 2006). |
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| (summation) | [DefinedSet:1121719] fumarylacetoacetase [mitochondrion] [Oryza sativa]
[EntityWithAccessionedSequence:1122849] fumarylacetoacetase [mitochondrion] [Oryza sativa]
[EntityWithAccessionedSequence:1125883] fumarylacetoacetate hydrolase [mitochondrion] [Oryza sativa]
[EntityWithAccessionedSequence:1129390] fumarylacetoacetase [mitochondrion] [Oryza sativa]
[Reaction:71181] FAH cleaves 4FAA [Homo sapiens] |
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No pathways have been reviewed or authored by This is the final step of tyrosine degradation. Fumarylaceto... (178797)