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BCKDK Mouse anti-Human, Clone: OTI11C9, Invitrogen™

Mouse Monoclonal Antibody

Manufacturer:  Invitrogen MA524992

Catalog No. PIMA524992


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Description

Description

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.
Specifications

Specifications

BCKDK
Monoclonal
0.8 mg/mL
PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3
O14874
BCKD-kinase, BCKDHKIN, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, branched chain alpha-ketoacid dehydrogenase kinase, branched-chain alpha-ketoacid dehydrogenase kinase
Mouse
IgG1
100 μL
-20° C, Avoid Freeze/Thaw Cycles
Primary
10295
Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
OTI11C9
Unconjugated
BCKDK
Liquid
BCKDK
Full length human recombit protein of BCKDK produced in HEK293T cell
Affinity Chromatography
RUO
Antibody
Monoclonal
Human
Documents
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