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Lipoamide Acyltransferase Component of Branched-Chain Alpha-Keto Acid Dehydrogenase Complex, Mitochondrial (DBT) ELISA Kit for Human

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS761050

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human DBT antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human DBT antibody was used as detection antibodies. The… More details

$405 / 48-Strip-Wells +1 FREE 8GB USB

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

Online Datasheet in PDF
Product Name
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT), ELISA Kit
Full Product Name
Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial ELISA Kit
Product Gene Name
DBT elisa kit
Product Synonym Names
DBT/BCATE2/BCKAD-E2/BCKADE2/E2/E2B/Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase/Dihydrolipoamide branched chain transacylase/Branched-chain alpha-keto acid dehydrogenase complex component E2/Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex
Species Reactivity
Human
Assay Type
Sandwich ELISA, Double Antibody
Samples
Serum, Plasma, Cell Culture Supernatant, cell or tissue lysate, Other liquid samples
Detection Range
0.313-20ng/ml
Sensitivity
0.188ng/ml
Preparation and Storage
Store entire kit at 2-8C for short-term. For longer-term, please store the microplate & standard at -20C, while the remaining reagents can be stored at 2-8C
Molecular Weight
53,487 Da
OMIM
248600
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Typical Testing Data of DBT elisa kit

Human DBT ELISA Kit – Cat# MBS761050

Typical Standard Curve of DBT elisa kit

Human DBT ELISA Kit, Typical Standard Curve – Cat# MBS761050

Other Notes

Small volumes of DBT elisa kit 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.

Searchable Terms for DBT purchase

MBS761050 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing DBT. The ELISA analytical biochemical technique of the MBS761050 kit is based on DBT antibody-DBT antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect DBT antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, DBT. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Additional Details

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

NCBI GI #
392494079
NCBI GeneID
1629
NCBI Accession #
NP_001909.3
UniProt Secondary Accession #
Q5VVL8; B2R811
UniProt Related Accession #
P11182
GenBank Nucleotide #
NM_001918.3
NCBI Official Full Name
lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
NCBI Official Synonym Full Names
dihydrolipoamide branched chain transacylase E2
NCBI Official Symbol
DBT
NCBI Official Synonym Symbols
E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2
UniProt Protein Name
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
UniProt Synonym Protein Names
52 kDa mitochondrial autoantigen of primary biliary cirrhosis
UniProt Gene Name
DBT
UniProt Synonym Gene Names
BCATE2; BCKAD-E2; BCKADE2
UniProt Entry Name
ODB2_HUMAN
NCBI Protein Information
lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

NCBI Summary for DBT

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008]
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UniProt Comments for DBT

DBT: 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 DBT are the cause of maple syrup urine disease type 2 (MSUD2). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. Belongs to the 2-oxoacid dehydrogenase family.

Protein type: Transferase; EC 2.3.1.168; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 1p31

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

Molecular Function: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity; ubiquitin protein ligase 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 DBT

1. Deletion in DBT 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

Products associated with DBT elisa kit

 Reference Product  PubMed Publications
 BCKDHB elisa kit  >27 publications with DBT and BCKDHB
 BCKDHA elisa kit  >25 publications with DBT and BCKDHA
 DLD elisa kit  >6 publications with DBT and DLD
 ACADM elisa kit  >1 publications with DBT and ACADM
 IVD elisa kit  >1 publications with DBT and IVD

Pathways associated with DBT elisa kit

 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 DBT elisa kit

 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >85 publications with DBT and Nervous System Diseases
 Maple Syrup Urine Disease Antibodies  >35 publications with DBT and Maple Syrup Urine Disease
 Necrosis Antibodies  >14 publications with DBT and Necrosis
 Adenocarcinoma Antibodies  >12 publications with DBT and Adenocarcinoma
 Weight Loss Antibodies  >10 publications with DBT and Weight Loss
 Liver Diseases Antibodies  >10 publications with DBT and Liver Diseases
 Inflammation Antibodies  >9 publications with DBT and Inflammation
 Hypertension Antibodies  >9 publications with DBT and Hypertension
 Abnormalities, Drug-Induced Antibodies  >7 publications with DBT and Abnormalities, Drug-Induced
 Fever Antibodies  >6 publications with DBT and Fever

Organs/Tissues associated with DBT elisa kit

 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >140 publications with DBT and Blood
 Brain Antibodies  >86 publications with DBT and Brain
 Liver Antibodies  >62 publications with DBT and Liver
 Heart Antibodies  >35 publications with DBT and Heart
 Skin Antibodies  >22 publications with DBT and Skin
 Bone Antibodies  >21 publications with DBT and Bone
 Kidney Antibodies  >20 publications with DBT and Kidney
 Adipose Tissue Antibodies  >14 publications with DBT and Adipose Tissue
 Mouth Antibodies  >13 publications with DBT and Mouth
 Connective Tissue Antibodies  >11 publications with DBT and Connective Tissue
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