Gene name: FAHD1

Uniprot entry:

Q6P587

Protein names:

Acylpyruvase FAHD1, mitochondrial (EC 3.7.1.5) (Fumarylacetoacetate hydrolase domain-containing protein 1) (FAH domain-containing protein 1) (Oxaloacetate decarboxylase) (OAA decarboxylase) (EC 4.1.1.112) (YisK-like protein)

Protein sequence:

1_MGIMA 6_ ASRPL 11_ SRFWE 16_ WGKNI 21_ VCVGR 26_ NYADH 31_ VREMR 36_ SAVLS 41_ EPVLF 46_ LKPST 51_ AYAPE 56_ GSPIL 61_ MPAYT 66_ RNLHH 71_ ELELG 76_ VVMGK 81_ RCRAV 86_ PEAAA 91_ MDYVG 96_ GYALC 101_ LDMTA 106_ RDVQD 111_ ECKKK 116_ GLPWT 121_ LAKSF 126_ TASCP 131_ VSAFV 136_ PKEKI 141_ PDPHK 146_ LKLWL 151_ KVNGE 156_ LRQEG 161_ ETSSM 166_ IFSIP 171_ YIISY 176_ VSKII 181_ TLEEG 186_ DIILT 191_ GTPKG 196_ VGPVK 201_ ENDEI 206_ EAGIH 211_ GLVSM 216_TFKVE

Protein annotations

Protein functions:

1: 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)