BCKDH kinase (BCKDK) (C-term) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	ELISA: 1/1,000. Western blotting: 1/100 - 1/500. Flow cytometry.
Reactivity	Human, Mouse
Host	Rabbit
Clonality	Polyclonal
Immunogen	This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the C-terminal region of human BCKDK.
Specificity	This antibody reacts to BCKDK.
Formulation	PBS with 0.09% (W/V) sodium azide State: Purified State: Liquid purified Ig
Concentration	lot specific
Purification	Protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS
Conjugation	Unconjugated
Storage Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	branched chain ketoacid dehydrogenase kinase
Database Link	Entrez Gene 10295 Human UniProt ID O14874
Background	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.
Synonyms	BCKD-kinase, BCKDHKIN
Reference Data

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