HADHB Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity. Belongs to the thiolase-like superfamily. Thiolase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC; Lipid Metabolism - fatty acid; Lipid Metabolism - fatty acid elongation in mitochondria; Transferase
Chromosomal Location of human Ortholog: 2p23.3
Cellular Component:  endoplasmic reticulum; mitochondrial envelope; mitochondrial inner membrane; mitochondrial nucleoid; mitochondrial outer membrane; mitochondrion
Molecular Function:  3-hydroxyacyl-CoA dehydrogenase activity; protein binding
Biological Process:  cardiolipin acyl-chain remodeling; fatty acid beta-oxidation
Disease: Trifunctional Protein Deficiency
Reference #:  P55084 (UniProtKB)
Alt. Names/Synonyms: 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; Beta-ketothiolase; ECHB; HADHB; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta; 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 A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit; MGC87480; mitochondrial trifunctional enzyme, beta subunit; mitochondrial trifunctional protein, beta subunit; MSTP029; MTPB; TP-beta; Trifunctional enzyme subunit beta, mitochondrial
Gene Symbols: HADHB
Molecular weight: 51,294 Da
Basal Isoelectric point: 9.45  Predict pI for various phosphorylation states
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Protein Structure Not Found.

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