Products

Western Blot (Transfected lysate)

Immunofluorescence (Transfected cell)

Flow Cytometry (Transfected cell)

Entrez GeneID

GeneBank Accession#

Protein Accession#

Gene Name

HADHA

Gene Alias

ECHA, GBP, HADH, LCHAD, MGC1728, MTPA, TP-ALPHA

Gene Description

hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit

Omim ID

Gene Ontology

Gene Summary

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. [provided by RefSeq

Other Designations

3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit|3-oxoacyl-CoA thiolase|78 kDa gastrin-binding protein|OTTHUMP00000122457|mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit|mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (Co