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.

2-enoyl-Coenzyme A (CoA) hydratase, beta subunit; 3-ketoacyl-CoA thiolase; 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit; Acetyl-CoA acyltransferase; bet; Beta-ketothiolase; hydroxyacyl-Co; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit; TP-beta; Trifunctional enzyme subunit beta, mitochondrial; ECHB; HADHB; MSTP029; MTPB; TP-BETA;

O46629, P55084

51kDa

Mouse monoclonal

Cow, Human, Mouse, Rat

WB 1:500-1:2000, IP 1:50-100

Store at -20 °C. Stable for 12 months from date of receipt.