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BCKDK Mouse anti-Human, Clone: OTI11C9, liquid, TrueMAB™ Non-distribution product as customer accommodation.

Mouse Monoclonal Antibody

Manufacturer:  OriGeneSupplier Diversity Partner TA500685

Catalog No. 50-167-1830


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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 ubiquitiously 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
O14874
BCKDKD, BDK
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 human BCKDK produced in HEK293T cell.
Affinity Chromatography
RUO
Antibody
Monoclonal
Human
Documents
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