BCKDHA Antibody - N-terminal regio : FITC (ARP56119 P050-FITC)

Branched chain keto acid dehydrogenase E1, alpha polypeptide

MSU, MSUD1, OVD1A, BCKDE1A

593

P12694

NP_000700

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

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

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 BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]) . The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331) . E1 is a thiamine pyrophosphate (TPP) -dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.[supplied by OMIM]. 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. PRIMARYREFSEQ_SPAN PRIMARY_IDENTIFIER PRIMARY_SPAN COMP 1-675 BI910860.1 12-686 676-1196 BG742673.1 80-600 1197-1731 BM702667.1 48-582 1732-1763 BE223026.1 1-32 c 1764-1781 BQ018849.1 1-18 c

UBC; CUL3; BCKDHB; BCKDK

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

IHC, WB

Affinity Purified

BCKDHA is supported by BioGPS gene expression data to be expressed in MCF7

0.5 mg/ml

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

Liquid. Purified antibody supplied in 1x PBS buffer.

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.

50kDa

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

Wet Ice

445

BCKDHA

BCKDHA

Rabbit

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

NM_000709

NVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILY

Alpha, mitochondrial