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Invitrogen™ BCKDK Polyclonal Antibody
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Catalog No. PIPA586367
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100 μL
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PIPA586367 100 μL
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Catalog No. PIPA586367 Supplier Invitrogen™ Supplier No. PA586367
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Description

Rabbit Polyclonal Antibody

Purity is > 95% by SDS-PAGE.

The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
TRUSTED_SUSTAINABILITY

Specifications

Antigen BCKDK
Applications Immunohistochemistry (Paraffin), Immunoprecipitation, Western Blot
Classification Polyclonal
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.2
Gene BCKDK
Gene Accession No. O14874, O55028, Q00972
Gene Alias [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; AI327402; BCKDHKIN; BCKDK; BCKDKD; BCKD-kinase; BDK; branched chain alpha-ketoacid dehydrogenase kinase; branched chain ketoacid dehydrogenase kinase; branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
Gene Symbols BCKDK
Host Species Rabbit
Immunogen Synthetic peptide, corresponding to amino acids 21-70 of Human BCKDK.
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 10295, 12041, 29603
Target Species Human, Mouse, Rat
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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