HADHA

HADHA
Identifiers
Aliases	HADHA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit, ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA
External IDs	OMIM: 600890 MGI: 2135593 HomoloGene: 152 GeneCards: HADHA
Gene location (Human) Chr. Chromosome 2 (human) Band n/a Start 26,190,635 bp End 26,244,726 bp
Gene location (Mouse) Chr. Chromosome 5 (mouse) Band n/a Start 30,118,304 bp End 30,155,162 bp
Gene ontology Molecular function • fatty-acyl-CoA binding • acetyl-CoA C-acyltransferase activity • protein complex binding • NAD binding • enoyl-CoA hydratase activity • acetyl-CoA C-acetyltransferase activity • long-chain-3-hydroxyacyl-CoA dehydrogenase activity • protein binding • catalytic activity • lyase activity • oxidoreductase activity • long-chain-enoyl-CoA hydratase activity • 3-hydroxyacyl-CoA dehydrogenase activity Cellular component • mitochondrion • mitochondrial inner membrane • mitochondrial nucleoid • mitochondrial fatty acid beta-oxidation multienzyme complex • extracellular matrix Biological process • lipid metabolic process • fatty acid metabolic process • response to insulin • fatty acid beta-oxidation • metabolic process • cardiolipin acyl-chain remodeling • oxidation-reduction process • response to drug Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	3030	97212
Ensembl	ENSG00000084754	ENSMUSG00000025745
UniProt	P40939	Q8BMS1
RefSeq (mRNA)	NM_000182	NM_178878
RefSeq (protein)	NP_000173	NP_849209
Location (UCSC)	Chr 2: 26.19 – 26.24 Mb	Chr 2: 30.12 – 30.16 Mb
PubMed search

3030

97212

ENSG00000084754

ENSMUSG00000025745

P40939

Q8BMS1

NM_000182

NM_178878

NP_000173

NP_849209

Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit is a protein that in humans is encoded by the HADHA gene.

HADHA is a 82.9 kDa protein composed of 763 amino acids.

The membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.

This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, and acetyl-CoA when NAD and CoASH are present. The alpha subunit catalyzes this reaction, and is attached to HADHB, which catalyzes the last step of the reaction.

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Wikipedia