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Invitrogen™ BCKDK Monoclonal Antibody (OTI11C9)
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Catalog No. PIMA524992
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100 μL
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PIMA524992 100 μL
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Catalog No. PIMA524992 Supplier Invitrogen™ Supplier No. MA524992
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Description

Mouse Monoclonal Antibody

BCKDK Monoclonal Antibody for Western Blot, ICC/IF, IHC (P)

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), Western Blot, Immunocytochemistry
Classification Monoclonal
Clone OTI11C9
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3
Gene BCKDK
Gene Accession No. O14874
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 Mouse
Immunogen Full length human recombinant protein of BCKDK produced in HEK293T cell.
Purification Method Affinity Chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 10295
Target Species Human
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG1
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