BCKDH kinase (BCKDK) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	WB: 1:1000, IHC: 1:50~100
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK.
Isotype	Ig
Formulation	Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide.
Concentration	lot specific
Purification	This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Predicted Protein Size	46360 Da
Gene Name	branched chain ketoacid dehydrogenase kinase
Database Link	NP_005872 Entrez Gene 12041 MouseEntrez Gene 29603 RatEntrez 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	BCKDKD; BDK
Reference Data
Protein Families	Druggable Genome, Protein Kinase

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