QuickView for HADHA (gene)

Gene Symbol:

HADHA Homo sapiens

Full name:

hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha

Synonyms:

ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, trifunctional enzyme subunit alpha, mitochondrial, 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit, 3-oxoacyl-CoA thiolase, 78 kDa gastrin-binding protein, gastrin-binding protein, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit, hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit, long-chain 2-enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit, mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit, mitochondrial trifunctional enzyme, alpha subunit, mitochondrial trifunctional protein, alpha subunit

Genomic Location:

Chr 2: 26267008-26321098

Description:

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 mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and eno...

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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 mitochondrial 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. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. 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.

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Orthologs:

Mus musculus ,  Rattus norvegicus ,  D. melanogaster ,  C. elegans ,  Macaca mulatta ,  Bos taurus ,  Gallus gallus ,  Pan troglodytes ,  Canis lupus familiaris ,  Danio rerio

External Links:

Entrez Gene

Transcription Factor
Binding Sites:

GTGCCTT_MIR506 ,  ARGGGTTAA_UNKNOWN

miRNA binding sites
(Source: TargetScan):

Predicted Gene Targets for miR-124a

Cytogenetic Map:

2p23.3

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