Anti-HADHB Antibody Picoband® Fluoro647 Conjugated

Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase, acetyl-CoA acyltransferase, or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene. 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.

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E.coli-derived human HADHB recombinant protein (Position: A18-H379) . Human HADHB shares 91.2% and 85.9% amino acid (aa) sequence identity with mouse and rat HADHB, respectively.

Downstream effector of TOR signaling pathway involved in osmotic stress response. Could be involved in the control of plant growth and development. Phosphorylates the ribosomal proteins P14, P16 and S6. Functions as a repressor of cell proliferation and required for maintenance of chromosome stability and ploidy levels through the RBR1-E2F pathway.

1. Carpenter, K., Pollitt, R. J., Middleton, B. Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria. Biochem. Biophys. Res. Commun. 183: 443-448, 1992. 2. Craig, I., Tolley, E., Bobrow, M. A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids. Birth Defects Orig. Art. Ser. XII (7) : 114-117, 1976. 3. Dagher, R., Massie, R., Gentil, B. J. MTP deficiency caused by HADHB mutations: pathophysiology and clinical manifestations. Molec. Genet. Metab. 133: 1-7, 2021.