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BCKDHA Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6464997

MBS6464997 is an antibody targeting BCKDHA. Also known as BCKDHA, CT (2-oxoisovalerate Dehydrogenase Subunit alpha, Mitochondrial, Branched-chain alpha-keto Acid Dehydrogenase E1 Component alpha Chain, BCKDH E1-alpha, BCKDE1A) (PE). Reactive in Human. Host:… More details

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Testing Data View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Product Overview

Online Datasheet in PDF
Product Name
BCKDHA, Polyclonal Antibody
Full Product Name
BCKDHA, CT (2-oxoisovalerate Dehydrogenase Subunit alpha, Mitochondrial, Branched-chain alpha-keto Acid Dehydrogenase E1 Component alpha Chain, BCKDH E1-alpha, BCKDE1A) (PE)
Product Gene Name
anti-BCKDHA antibody
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).
Purity/Purification
Purified by Ammonium Sulfate Precipitation.
Specificity
Recognizes human BCKDHA.
Preparation and Storage
Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Molecular Weight
50,790 Da
OMIM
248600
3D Structure
ModBase 3D Structure for P12694
Immunogen
Synthetic peptide selected from the C-terminal region of human BCKDHA (KLH).
Conjugate
PE
Note
Preservative Free

Product Categories/Family for anti-BCKDHA antibody

Antibodies; Protein Kinase Substrates
Applications Tested/Suitable for anti-BCKDHA antibody
Western Blot (WB), ELISA (EIA)
Application Notes for anti-BCKDHA antibody
WB: 1:50-1:100
ELISA: 1:1,000
Applications are based on unconjugated antibody.

Western Blot (WB) of anti-BCKDHA antibody

Western blot analysis of BCKDHA Antibody (C-term) in Jurkat cell line lysates (35ug/lane). BCKDHA (arrow) was detected using the purified Pab.
Rabbit Anti-BCKDHA Polyclonal Antibody – Cat# MBS6464997

Immunohistochemistry (IHC) of anti-BCKDHA antibody

Formalin-fixed and paraffin-embedded human lung carcinoma reacted with BCKDHA Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.
Rabbit Anti-BCKDHA Polyclonal Antibody, Immunohistochemistry (IHC) – Cat# MBS6464997

Flow Cytometry (FC/FACS) of anti-BCKDHA antibody

BCKDHA Antibody (C-term) flow cytometric analysis of Jurkat cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.
Rabbit Anti-BCKDHA Polyclonal Antibody, Flow Cytometry (FC/FACS) – Cat# MBS6464997

Product References and Citations for anti-BCKDHA antibody

1. Flaschker, N., et.al., J. Inherit. Metab. Dis. 30(6), 903-909 (2007).

Other Notes

Small volumes of anti-BCKDHA 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 BCKDHA. It may not necessarily be applicable to this product.

NCBI GI #
11386135
NCBI GeneID
593
NCBI Accession #
NP_000700.1
UniProt Primary Accession #
P12694
UniProt Secondary Accession #
Q16034; Q16472; B4DP47; E7EW46
UniProt Related Accession #
P12694
GenBank Nucleotide #
NM_000709.3
NCBI Official Full Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Official Symbol
BCKDHA
NCBI Official Synonym Symbols
MSU; MSUD1; OVD1A; BCKDE1A
UniProt Protein Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
UniProt Synonym Protein Names
Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha
UniProt Gene Name
BCKDHA
UniProt Synonym Gene Names
BCKDE1A; BCKDH E1-alpha
UniProt Entry Name
ODBA_HUMAN
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 2-oxoisovalerate dehydrogenase (lipoamide); BCKDH E1-alpha; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain

NCBI Summary for BCKDHA

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]
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UniProt Comments for BCKDHA

BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation

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

Chromosomal Location of Human Ortholog: 19q13.1-q13.2

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

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

Biological Process: branched chain family amino acid catabolic process

Disease: Maple Syrup Urine Disease
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Research Articles on BCKDHA

1. Data from infant/her heterozygous parents (first cousins) suggest homozygous mutation (S144I) in BCKDHA can result in maple syrup urine disease (IA); molecular modeling suggests this missense mutation in exon 4 affects protein stability. [CASE STUDY]

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

Products associated with anti-BCKDHA antibody

 Reference Product  PubMed Publications
 BCKDHB antibody  >22 publications with BCKDHA and BCKDHB
 DBT antibody  >21 publications with BCKDHA and DBT
 DLD antibody  >4 publications with BCKDHA and DLD
 IVD antibody  >1 publications with BCKDHA and IVD
 BCAT2 antibody  >1 publications with BCKDHA and BCAT2

Pathways associated with anti-BCKDHA 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
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram

Diseases associated with anti-BCKDHA antibody

 Disease Name  Pubmed Publications
 Maple Syrup Urine Disease Antibodies  >28 publications with BCKDHA and Maple Syrup Urine Disease
 Edema Antibodies  >1 publications with BCKDHA and Edema
 Liver Cirrhosis Antibodies  >1 publications with BCKDHA and Liver Cirrhosis
 Liver Failure Antibodies  >1 publications with BCKDHA and Liver Failure
 Coma Antibodies  >1 publications with BCKDHA and Coma
 Fibrosis Antibodies  >1 publications with BCKDHA and Fibrosis
 Fatty Liver Antibodies  >1 publications with BCKDHA and Fatty Liver

Organs/Tissues associated with anti-BCKDHA antibody

 Organ/Tissue Name  Pubmed Publications
 Liver Antibodies  >6 publications with BCKDHA and Liver
 Blood Antibodies  >6 publications with BCKDHA and Blood
 Muscle Antibodies  >4 publications with BCKDHA and Muscle
 Heart Antibodies  >2 publications with BCKDHA and Heart
 Adipose Tissue Antibodies  >2 publications with BCKDHA and Adipose Tissue
 Kidney Antibodies  >2 publications with BCKDHA and Kidney
 Intestine Antibodies  >2 publications with BCKDHA and Intestine
 Lung Antibodies  >2 publications with BCKDHA and Lung
 Connective Tissue Antibodies  >1 publications with BCKDHA and Connective Tissue
 Ovary Antibodies  >1 publications with BCKDHA and Ovary
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