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BCKDHB Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS767066

MBS767066 is an antibody targeting BCKDHB. Also known as BCKDHB Rabbit Polyclonal. Reactive in Human, Mouse, Rat. Host: Rabbit. More details

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Product Documentation
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Sample Manual Insert View PDF
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Testing Data View PDF
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Certificate of Analysis (COA) View PDF
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QC Report View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Protocol View PDF

Product Overview

Product Name
BCKDHB, Polyclonal Antibody
Full Product Name
BCKDHB Rabbit Polyclonal
Product Gene Name
anti-BCKDHB antibody
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Form/Format
Liquid
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
Molecular Weight
23,550 Da
OMIM
248600
Immunogen
Branched chain keto acid dehydrogenase E1, beta polypeptide
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Applications Tested/Suitable for anti-BCKDHB antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Immunohistochemistry (IHC) of anti-BCKDHB antibody

Immunohistochemistry of paraffin-embedded human renal cell carcinoma tissue slide using MBS767066 (BCKDHB Antibody) at dilution of 1:50
Rabbit Anti-BCKDHB Polyclonal Antibody – Cat# MBS767066

Western Blot (WB) of anti-BCKDHB antibody

HEK-293 cells were subjected to SDS PAGE followed by western blot with MBS767066 (BCKDHB Antibody) at dilution of 1:600
Rabbit Anti-BCKDHB Polyclonal Antibody, Western Blot (WB) – Cat# MBS767066

Other Notes

Small volumes of anti-BCKDHB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Additional Details

NCBI/Uniprot data below describe general gene information for BCKDHB. It may not necessarily be applicable to this product.

NCBI GI #
922312244
NCBI GeneID
594
NCBI Accession #
ALA62332.1
UniProt Secondary Accession #
Q5T2J3; Q9BQL0
UniProt Related Accession #
P21953
NCBI Official Full Name
BCKDHB protein
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1, beta polypeptide
NCBI Official Symbol
BCKDHB
NCBI Official Synonym Symbols
E1B; BCKDE1B; BCKDH E1-beta
UniProt Protein Name
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
UniProt Synonym Protein Names
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta
UniProt Gene Name
BCKDHB
UniProt Synonym Gene Names
BCKDE1B; BCKDH E1-beta
UniProt Entry Name
ODBB_HUMAN
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

NCBI Summary for BCKDHB

This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]
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UniProt Comments for BCKDHB

BCKDHB: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.

Protein type: EC 1.2.4.4; Mitochondrial; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 6q14.1

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion

Molecular Function: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; protein binding

Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process

Disease: Maple Syrup Urine Disease
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Research Articles on BCKDHB

1. Mutations in BCKDHB gene is associated with maple syrup urine disease.

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Pathways associated with anti-BCKDHB antibody

 Products by Pathway  Pathway Diagram
 2-oxobutanoate Degradation Pathway antibodies  2-oxobutanoate Degradation Pathway Diagram
 2-oxobutanoate Degradation I Pathway antibodies  2-oxobutanoate Degradation I Pathway Diagram
 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway antibodies  2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway Diagram
 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway antibodies  2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway Diagram
 Branched-chain Amino Acid Catabolism Pathway antibodies  Branched-chain Amino Acid Catabolism Pathway Diagram
 Glyoxylate Metabolism And Glycine Degradation Pathway antibodies  Glyoxylate Metabolism And Glycine Degradation Pathway Diagram
 L-isoleucine Degradation I Pathway antibodies  L-isoleucine Degradation I Pathway Diagram
 L-leucine Degradation I Pathway antibodies  L-leucine Degradation I Pathway Diagram
 L-threonine Degradation V Pathway antibodies  L-threonine Degradation V Pathway Diagram
 L-valine Degradation I Pathway antibodies  L-valine Degradation I Pathway Diagram

Diseases associated with anti-BCKDHB antibody

 Disease Name  Pubmed Publications
 Maple Syrup Urine Disease Antibodies  >31 publications with BCKDHB and Maple Syrup Urine Disease
 Edema Antibodies  >1 publications with BCKDHB and Edema
 Necrosis Antibodies  >1 publications with BCKDHB and Necrosis

Organs/Tissues associated with anti-BCKDHB antibody

 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >9 publications with BCKDHB and Blood
 Liver Antibodies  >3 publications with BCKDHB and Liver
 Brain Antibodies  >3 publications with BCKDHB and Brain
 Heart Antibodies  >1 publications with BCKDHB and Heart
 Intestine Antibodies  >1 publications with BCKDHB and Intestine
 Lung Antibodies  >1 publications with BCKDHB and Lung
 Kidney Antibodies  >1 publications with BCKDHB and Kidney
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