BCKDK Mouse anti-Human, Clone: OTI11C9, liquid, TrueMAB
Mouse Monoclonal Antibody
Manufacturer: OriGene TA500685
DescriptionThe 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.
|PBS with 1% BSA, 50% glycerol and 0.02% sodium azide|
|-20° C, Avoid Freeze/Thaw Cycles|
|Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot|
|Full length human recombit protein of human BCKDK produced in HEK293T cell.|
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