Anti-DBT Antibody Picoband®

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.

Boster Bio Anti-DBT Antibody Picoband® catalog # A01145-2. Tested in WB, ICC/IF, IP, Flow Cytometry, ELISA applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

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.

WB, ICC/IF, IP, Flow Cytometry, ELISA

Immunogen affinity purified.

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Lyophilized

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

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. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

https://www.bosterbio.com/msds?sku=A01145-2

53 kDa

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 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.