DBT Antibody - N-terminal regio : HRP (ARP51995_P050-HRP)

Dihydrolipoamide branched chain transacylase E2

E2, E2B, BCATE2, BCKADE2, BCKAD-E2, BCKDH-E2, BCOADC-E2

1629

P11182

NP_001909

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Yeast, Zebrafish

The immunogen is a synthetic peptide directed towards the N terminal region of human DBT

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) .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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

HUWE1; UBC; PARK2; IFIT2; ABCC1; DLD; AGO4; AGO3; SRRM2; USP16; ADI1

HRP: Horseradish Peroxidase

Polyclonal Antibody

WB, IHC

Affinity Purified

There is BioGPS gene expression data showing that DBT is expressed in HT1080

0.5 mg/ml

Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Yeast: 83%; Zebrafish: 93%

Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

46kDa

Flaschker, N., (2007) J. Inherit. Metab. Dis. 30 (6), 903-909

Wet Ice

482

DBT

DBT

Rabbit

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

NM_001918

NYVCFFGYPSFKYSHPHHFLKTTAALRGQVVQFKLSDIGEGIREVTVKEW