Anti-BCKDK (C-term T340) Antibody

Product Description

Specifications

Size	400 µL
Estimated Lead Time	1-2 weeks
Species	Human, Mouse
Host Species	Rabbit
Accession Number	O14874
Gene Id	10295
Gene Symbols	BCKDK
Protein Name / Synonyms	BCKDK (C-term T340)
Clonality	Polyclonal
Preparation	This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 325-356 amino acids from the C-terminal region of human BCKDK.
Concentration (lot specific)	2.0 mg/ml
Recommended Applications	Western Blotting
Shipping Type	Blue ice
Storage	-20°C

Description

Introduction

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.

Specificity

Predicted Reactivity

Rat

Other Names

BCKDK; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; Branched-chain alpha-ketoacid dehydrogenase kinase

NCBI Accession #

NP_001116429.1;NP_005872.2

Other Accession #

Q00972

Format

Type

Purified Rabbit Polyclonal Antibody (Pab)

Calculated Molecular Weight (Da)

46360

Recommended Dilutions

WB: 1:1000

	The anti-BCKDK Pab (Cat# 102-17121) is used in Western blot to detect BCKDK in mouse intestine tissue lysate (Lane 1) and Hela cell lysate (Lane 2).
	Western blot analysis of BCKDK (arrow) using rabbit polyclonal BCKDK Antibody (C-term T340) (RB05257).293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2) (Origene Technologies).

Antigen Source

HUMAN

Storage/Stability

2-8°C (short-term); -20°C (long-term)

Expiration:

12 months from the date of shipment when stored properly.

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