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, hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
External IDs	OMIM: 600890; MGI: 2135593; HomoloGene: 152; GeneCards: HADHA; OMA:HADHA - orthologs
Gene location (Human)
Chr.	Chromosome 2 (human)
End	26,244,672 bp
Gene location (Mouse)
Chr.	Chromosome 5 (mouse)
End	30,360,160 bp
RNA expression pattern
	Top expressed in
	jejunal mucosa; ; gastrocnemius muscle; ; left ventricle; ; apex of heart; ; duodenum; ; Skeletal muscle tissue of rectus abdominis; ; right auricle of heart; ; mucosa of transverse colon; ; muscle of thigh; ; rectum;
	Top expressed in
	myocardium of ventricle; ; right ventricle; ; cardiac muscles; ; brown adipose tissue; ; muscle of thigh; ; genital tubercle; ; extraocular muscle; ; soleus muscle; ; epithelium of small intestine; ; digastric muscle;
	More reference expression data
	n/a
Gene ontology
Molecular function	fatty-acyl-CoA binding; acetyl-CoA C-acyltransferase activity; protein-containing 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 metabolism; fatty acid metabolic process; response to insulin; fatty acid beta-oxidation; metabolism; cardiolipin acyl-chain remodeling;
	Sources:Amigo / QuickGO
Orthologs
	3030
	97212
	ENSG00000084754
	ENSMUSG00000025745
	P40939
	Q8BMS1
	NM_000182
	NM_178878
	NP_000173
	NP_849209
	Wikidata
View/Edit Human	View/Edit Mouse

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. Mutations in HADHA have been associated with trifunctional protein deficiency or long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.