FAHD1

FAHD1
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesFAHD1, C16orf36, YISKL, fumarylacetoacetate hydrolase domain containing 1
External IDsMGI: 1915886 HomoloGene: 6774 GeneCards: FAHD1
EC number4.1.1.3
Orthologs
SpeciesHumanMouse
Entrez

81889

68636

Ensembl

ENSG00000180185

ENSMUSG00000045316

UniProt

Q6P587

Q8R0F8

RefSeq (mRNA)

NM_031208
NM_001018104
NM_001142398

NM_023480

RefSeq (protein)

NP_001018114
NP_001135870
NP_112485

NP_075969

Location (UCSC)Chr 16: 1.83 – 1.84 MbChr 17: 24.85 – 24.85 Mb
PubMed search[1][2]
Wikidata
View/Edit HumanView/Edit Mouse

Fumarylacetoacetate hydrolase domain-containing protein 1, also known as FLJ36880 protein, is an enzyme that in humans is encoded by the FAHD1 gene on chromosome 16.[3]

Structure

The FAHD1 gene encodes for a 24-kDa protein that is localized to the mitochondrion and belongs to the fumarylacetoacetate hydrolase family of proteins.[4] The structure of FAHD1 has been resolved using X-ray crystallography at 2.2-Å resolution. The overall structure is similar to the C-terminal domain of the bifunctional enzyme HpcE from Escherichia coli C, fumarylacetoacetate hydrolase from Mus musculus and to YcgM (Apc5008) from E. coli 1262.[4] A number of conserved amino acids including Asp-102 and Arg-106 of FAHD1 appear to be important for its catalytic activity.[5]

Function

The FAHD1 protein has been shown to function as an oxaloacetate decarboxylase in eukaryotes.[6] The FAHD1 protein probably also functions as an acylpyruvase, having been shown to catalyze the hydrolysis of acetylpyruvate and fumarylpyruvate in in vitro experiments.[4] Mg(2+) was required for maximal enzyme activity.[5]

References

  1. "Human PubMed Reference:".
  2. "Mouse PubMed Reference:".
  3. "Entrez Gene: FAHD1 fumarylacetoacetate hydrolase domain containing 1".
  4. 1 2 3 Manjasetty BA, Niesen FH, Delbrück H, Götz F, Sievert V, Büssow K, Behlke J, Heinemann U (2004). "X-ray structure of fumarylacetoacetate hydrolase family member Homo sapiens FLJ36880". Biol. Chem. 385 (10): 935–42. PMID 15551868. doi:10.1515/BC.2004.122.
  5. 1 2 Pircher H, Straganz GD, Ehehalt D, Morrow G, Tanguay RM, Jansen-Dürr P (2011). "Identification of human fumarylacetoacetate hydrolase domain-containing protein 1 (FAHD1) as a novel mitochondrial acylpyruvase". J. Biol. Chem. 286 (42): 36500–8. PMC 3196145Freely accessible. PMID 21878618. doi:10.1074/jbc.M111.264770.
  6. Pircher H, von Grafenstein S, Diener T, Metzger C, Albertini E, Taferner A, Unterluggauer H, Kramer C, Liedl KR, Jansen-Dürr P (2015). "Identification of FAH domain-containing protein 1 (FAHD1) as oxaloacetate decarboxylase". J. Biol. Chem. 290 (11): 6755–62. PMC 4358102Freely accessible. PMID 25575590. doi:10.1074/jbc.M114.609305.

Further reading


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