HADHB, Official Symbol HADHBprovided by HGNC Official Full Name hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit betaprovided by HGNC Primary source HGNC:HGNC:4803 See related Ensembl:ENSG00000138029 MIM:143450; AllianceGenome:HGNC:4803 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as ECHB; MTPB; MTPD; MTPD2; MSTP029; TP-BETA Summary This gene 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. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013] Expression Ubiquitous expression in heart (RPKM 140.9), duodenum (RPKM 87.4) and 25 other tissues See more Orthologs mouse all