Anti-HADHA Antibody Picoband® Fluoro488 Conjugated

Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit is a protein that in humans is encoded by the HADHA gene. This gene encodes the alpha 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 alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. 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.

E.coli-derived human HADHA recombinant protein (Position: R20-N758) . Human HADHA shares 86.6% and 95.2% amino acid (aa) sequence identity with mouse and rat HADHA, respectively.

1. Brackett, J. C., Sims, H. F., Rinaldo, P., Shapiro, S., Powell, C. K., Bennett, M. J., Strauss, A. W. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency. J. Clin. Invest. 95: 2076-2082, 1995. 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. Dionisi Vici, C., Burlina, A. B., Bertini, E., Bachmann, C., Mazziotta, M. R. M., Zacchello, F., Sabetta, G., Hale, D. E. Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. J. Pediat. 118: 744-746, 1991.