FAHD1
entrez
81889
canonical
uniprot
Q6P587
canonical
ensembl
ENSG00000180185
resolver
ensp
ENSP00000372112
resolver
ensp
ENSP00000372114
resolver
ensp
ENSP00000398053
resolver
ensp
ENSP00000718238
resolver
genesymbol
FAHD1
resolver
genesymbol_syn
C16orf36
resolver
genesymbol_syn
YISKL
resolver
hgnc
HGNC:14169
resolver
uniprot
B1AK40
resolver
uniprot
B1AK41
resolver
uniprot
Q6FIC7
resolver
uniprot
Q96RY1
resolver
uniprot
Q9H0N6
resolver
uniprot_entry
FAHD1_HUMAN
resolver
uniprot_sec
B1AK40
resolver
uniprot_sec
B1AK41
resolver
uniprot_sec
Q6FIC7
resolver
uniprot_sec
Q96RY1
resolver
uniprot_sec
Q9H0N6
resolver
activity_regulation
ACTIVITY REGULATION: Oxaloacetate decarboxylation is competitively inhibited by oxalate. {ECO:0000250|UniProtKB:Q8R0F8}.
uniprot
proteins
amino_acid_sequence
MAASRPLSRFWEWGKNIVCVGRNYADHVREMRSAVLSEPVLFLKPSTAYAPEGSPILMPAYTRNLHHELELGVVMGKRCRAVPEAAAMDYVGGYALCLDMTARDVQDECKKKGLPWTLAKSFTASCPVSAFVPKEKIPDPHKLKLWLKVNGELRQEGETSSMIFSIPYIISYVSKIITLEEGDIILTGTPKGVGPVKENDEIEAGIHGLVSMTFKVEKPEY
uniprot
proteins
ec_number
3.7.1.5
uniprot
proteins
ec_number
4.1.1.112
uniprot
proteins
ec_number
5.3.2.2
uniprot
proteins
function
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). {ECO:0000269|PubMed:21878618, ECO:0000269|PubMed:25575590, ECO:0000269|PubMed:30348641, ECO:0000269|PubMed:38287013}.
uniprot
proteins
mass_dalton
24542
uniprot
proteins
mutagenesis
MUTAGEN 27; /note="H->A: Loss of acetylpyruvate hydrolase activity, while moderate decrease in oxaloacetate decarboxylase activity."; /evidence="ECO:0000269|PubMed:25575590, ECO:0000269|PubMed:30348641"; MUTAGEN 30; /note="E->A: Loss of acetylpyruvate hydrolase activity, while moderate decrease in oxaloacetate decarboxylase activity."; /evidence="ECO:0000269|PubMed:25575590, ECO:0000269|PubMed:30348641"; MUTAGEN 99..103; /note="DMTAR->AMTAA: Loss of oxaloacetate tautomerase, oxaloacetate decarboxylase and acetylpyruvate hydrolase activities."; /evidence="ECO:0000269|PubMed:38287013"; MUTAGEN 120; /note="K->A: Loss of both oxaloacetate decarboxylase and acetylpyruvate hydrolase activity."; /evidence="ECO:0000269|PubMed:30348641
uniprot
proteins
protein_family
FAH family
uniprot
proteins
pubmed
11157797
uniprot
proteins
pubmed
11230166
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15551868
uniprot
proteins
pubmed
15616553
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
21878618
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25575590
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
30348641
uniprot
proteins
pubmed
38287013
uniprot
proteins
sequence_length
221
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000269|PubMed:21878618}. Cytoplasm, cytosol {ECO:0000269|PubMed:21878618}.
uniprot
proteins