Anti-DBT Antibody Picoband® fluoro488 Vervoeging

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.

DBT; BCATE2; Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; EC 2.3.1.168; 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Branched chain 2-oxo-acid dehydrogenase complex component E2; BCOADC-E2; Branched-chain alpha-keto acid dehydrogenase complex component E2; BCKAD-E2; BCKADE2; Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; Dihydrolipoamide branched chain transacylase; Dihydrolipoyllysine-residue; 2-methylpropanoyltransferase

DBT

1629

P11182

Human, Mouse, Rat

No cross-reactivity with other proteins

E.coli-derived human DBT recombinant protein (Position: K46-R462) . Human DBT shares 89.7% amino acid (aa) sequence identity with mouse DBT.

Flow Cytometry

Immunogen affinity purified.

Liquid

1. Bogenhagen, D. F., Rousseau, D., Burke, S. The layered structure of human mitochondrial DNA nucleoids. J. Biol. Chem. 283: 3665-3675, 2008. 2. Chi, C.-S., Tsai, C.-R., Chen, L.-H., Lee, H.-F., Mak, B. S.-C., Yang, S.-H., Wang, T.-Y., Shu, S.-G., Chen, C.-H. Maple syrup urine disease in the Austronesian aboriginal tribe Paiwan of Taiwan: a novel DBT (E2) gene 4.7 kb founder deletion caused by a nonhomologous recombination between LINE-1 and Alu and the carrier-frequency determination. Europ. J. Hum. Genet. 11: 931-936, 2003. 3. Chuang, D. T., Fisher, C. W., Lau, K. S., Griffin, T. A., Wynn, R. M., Cox, R. P. Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex. Molec. Biol. Med. 8: 49-63, 1991.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

53 kDa

6

Dihydrolipoamide branched chain transacylase E2

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.