XB-FEAT-959218

hadha

This is the community wiki page for the gene hadha please feel free to add any information that is relevant to this gene that is not already captured elsewhere in Xenbase.

nomenclature changes

09/24/2017 Human name has changed for Entrez Gene: 3030. From hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit to hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha

summary from NCBI

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, Jul 2008]