Protein Name:
Oxaloacetate tautomerase FAHD1, mitochondrial
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UniprotKB/SwissProt ID: Q6P587 (Q6P587)
Gene Name:
FAHD1
Organism: Homo sapiens (Human)
Function: Tautomerase that converts enol-oxaloacetate, a strong inhibitor of succinate dehydrogenase, to the physiological keto form of oxaloacetate (PubMed:38287013). It is thereby required to maximize aerobic respiration efficiency by preventing succinate dehydrogenase inhibition (PubMed:38287013). Also acts as a weak oxaloacetate decarboxylase (ODx), catalyzing the decarboxylation of oxaloacetate (OAA) to pyruvate and CO(2), and as such is likely a regulatory enzyme in the TCA cycle (PubMed:25575590, PubMed:30348641). Also displays acylpyruvase activity, being able to hydrolyze acetylpyruvate and fumarylpyruvate in vitro (PubMed:21878618, PubMed:30348641). Exhibits only a weak hydrolase activity on methylacetopyruvate and acetylacetone, and no activity toward acetoacetyl-CoA (PubMed:21878618)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion. Cytoplasm, cytosol
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Graphical Visualization of S-nitrosylation Sites:
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| InterPro ID |
Domain Name |
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IPR011234
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Fumarylacetoacetase-like_C |
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IPR036663
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Fumarylacetoacetase_C_sf |
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The S-nitrosylation sites of Q6P587
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
109 |
DMTARDVQDE C KKKGLPWTLA |
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| 2 |
126 |
WTLAKSFTAS C PVSAFVPKEK |
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| 3 |
19 |
RFWEWGKNIV C VGRNYADHVR |
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| 4 |
97 |
AMDYVGGYAL C LDMTARDVQD |
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