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Product Details
TA500685
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG1
Class
Monoclonal
Type
Antibody
Clone
OTI11C9
Immunogen
Full length human recombinant protein of human BCKDK produced in HEK293T cell.
Conjugate
Form
Liquid
Concentration
0.8 mg/mL
Purification
Affinity chromatography
Storage buffer
PBS with 1% BSA, 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Ambient (domestic); Wet ice (international)
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKD-kinase; BCKDH kinase; BCKDHKIN; branched chain alpha-ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
Gene Aliases: BCKDK; BCKDKD; BDK
UniProt ID: (Human) O14874
Entrez Gene ID: (Human) 10295
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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