HADHB Products

Description

HADHB encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. [provided by RefSeq]

Bioinformatics

Entrez	3032 Human
Uniprot	NM_000183 Human NP_000174 Human NP_000174. Human NP_000174.1 Human P55084 Human
Product By Gene ID	3032
Alternate Names	2-enoyl-Coenzyme A (CoA) hydratase, beta subunit 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein acetyl-CoA acyltransferase beta subunit beta-ketothiolase EC 2.3.1 EC 2.3.1.16 ECHB hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase(trifunctional protein), beta subunit hydroxyacyl-Coenzyme A (CoA) dehydrogenase, beta subunit hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme Athiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit MGC87480 mitochondrial trifunctional enzyme, beta subunit mitochondrial trifunctional protein, beta subunit MTPB TP-beta TP-BETA trifunctional enzyme subunit beta, mitochondrial