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Product Description

Specifications

Size	400 µl
Protein Name / Synonyms	BCKDHB (N-term)
Accession Number	P21953
Gene Symbols	BCKDHB
Gene Id	594
Concentration (lot specific)	0.450 mg/ml
Preparation	This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
Host Species	Rabbit
Clonality	Polyclonal
Species	Human

Introduction

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.

Specificity

Other Names

BCKDHB; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain

NCBI Accession #

NP_000047.1;NP_898871.1

Other Accession #

NP_000047.1

Format

Type

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Calculated Molecular Weight (Da)

43122

Recommended Dilutions

WB: 1:1000; IHC: 1:50-100; FC: 1:10-50; IF: 1:10-50

	BCKDHB Antibody (N-term) (Cat# 102-10747) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow).
	BCKDHB Antibody (N-term) (Cat# 102-10747) immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BCKDHB Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.
	Western blot analysis of BCKDHB (arrow) using rabbit polyclonal BCKDHB Antibody (N-term) (Cat# 102-10747). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the BCKDHB gene.
	BCKDHB Antibody (N-term) (Cat# 102-10747) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.
	Confocal immunofluorescent analysis of BCKDHB Antibody (N-term)(Cat# 102-10747) with Hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).