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DBT recombinant protein :: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT) Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS948109 DBT recombinant protein
Unit / Price
0.05 mg (E-Coli)  /  $835 +1 FREE 8GB USB
0.05 mg (Baculovirus)  /  $1,065 +1 FREE 8GB USB
0.2 mg (E-Coli)  /  $1,120 +1 FREE 8GB USB
0.5 mg (E-Coli)  /  $1,235 +1 FREE 8GB USB
0.2 mg (Yeast)  /  $1,260 +1 FREE 8GB USB
0.05 mg (Mammalian-Cell)  /  $1,315 +1 FREE 8GB USB
0.1 mg (Baculovirus)  /  $1,350 +1 FREE 8GB USB
0.5 mg (Yeast)  /  $1,425 +1 FREE 8GB USB
1 mg (E-Coli)  /  $1,755 +2 FREE 8GB USB
0.5 mg (Baculovirus)  /  $1,940 +2 FREE 8GB USB
0.1 mg (Mammalian-Cell)  /  $1,985 +2 FREE 8GB USB
1 mg (Yeast)  /  $2,165 +2 FREE 8GB USB
1 mg (Baculovirus)  /  $2,555 +3 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT), Recombinant Protein

 Also Known As   

Recombinant Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT)

 Product Gene Name   

DBT recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence Positions    62-482aa, Full Length of Mature Protein
 Sequence    GQVVQFKLSD IGEGIREVTV KEWYVKEGDT VSQFDSICEV QSDKASVTIT SRYDGVIKKL YYNLDDIAYV GKPLVDIETE ALKDSEEDVV ETPAVSHDEH THQEIKGRKT LATPAVRRLA MENNIKLSEV VGSGKDGRIL KEDILNYLEK QTGAILPPSP KVEIMPPPPK PKDMTVPILV SKPPVFTGKD KTEPIKGFQK AMVKTMSAAL KIPHFGYCDE IDLTELVKLR EELKPIAFAR GIKLSFMPFF LKAASLGLLQ FPILNASVDE NCQNITYKAS HNIGIAMDTE QGLIVPNVKN VQICSIFDIA TELNRLQKLG SVGQLSTTDL TGGTFTLSNI GSIGGTFAKP VIMPPEVAIG ALGSIKAIPR FNQKGEVYKA QIMNVSWSAD HRVIDGATMS RFSNLWKSYL ENPAFMLLDL K
 OMIM    248600
 3D Structure    ModBase 3D Structure for P11182
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 Host    E Coli or Yeast or Baculovirus or Mammalian Cell
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 Purity/Purification    >85% (SDS-PAGE) (lot specific)
 Form/Format    Liquid containing glycerol
 Tag Information    This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.
 Sterility    Sterile filter available upon request.
 Endotoxin    Low endotoxin available upon request.
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 Species    Human
 Storage Buffer    Tris-based buffer, 50% glycerol
 Tag Information    Tag type will be determined during the manufacturing process
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 Preparation and Storage    Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of DBT recombinant protein 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.
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Related Product Information for DBT recombinant protein

   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.
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 [Other Products]
 NCBI GenBank Nucleotide #    NM_001918.3 [Other Products]
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 UniProt Primary Accession #    P11182 [Other Products]
 UniProt Secondary Accession #    Q5VVL8; B2R811 [Other Products]
 UniProt Related Accession #    P11182 [Other Products]
 Molecular Weight    53,487 Da
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 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 [Similar Products]
 NCBI Official Synonym Symbols   
E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2
[Similar Products]
 NCBI Protein Information    lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
 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 [Similar Products]
 UniProt Synonym Gene Names    BCATE2; BCKAD-E2; BCKADE2 [Similar Products]
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 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    The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
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 Research Articles on DBT    1. The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved.
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 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.
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Products associated with DBT recombinant proteinPathways associated with DBT recombinant protein
 Reference Product  PubMed Publications
 BCKDHB recombinant protein  >23 publications with DBT and BCKDHB
 BCKDHA recombinant protein  >21 publications with DBT and BCKDHA
 DLD recombinant protein  >4 publications with DBT and DLD
 IVD recombinant protein  >1 publications with DBT and IVD
 PER1 recombinant protein  >1 publications with DBT and PER1
 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 DBT recombinant proteinOrgans/Tissues associated with DBT recombinant protein
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >78 publications with DBT and Nervous System Diseases
 Brain Diseases Antibodies  >58 publications with DBT and Brain Diseases
 Maple Syrup Urine Disease Antibodies  >30 publications with DBT and Maple Syrup Urine Disease
 Heart Diseases Antibodies  >23 publications with DBT and Heart Diseases
 Necrosis Antibodies  >13 publications with DBT and Necrosis
 Adenocarcinoma Antibodies  >11 publications with DBT and Adenocarcinoma
 Liver Diseases Antibodies  >10 publications with DBT and Liver Diseases
 Weight Loss Antibodies  >9 publications with DBT and Weight Loss
 Abnormalities, Drug-Induced Antibodies  >7 publications with DBT and Abnormalities, Drug-Induced
 Inflammation Antibodies  >6 publications with DBT and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >125 publications with DBT and Blood
 Brain Antibodies  >63 publications with DBT and Brain
 Liver Antibodies  >60 publications with DBT and Liver
 Heart Antibodies  >29 publications with DBT and Heart
 Bone Antibodies  >19 publications with DBT and Bone
 Skin Antibodies  >19 publications with DBT and Skin
 Kidney Antibodies  >17 publications with DBT and Kidney
 Mouth Antibodies  >13 publications with DBT and Mouth
 Adipose Tissue Antibodies  >12 publications with DBT and Adipose Tissue
 Connective Tissue Antibodies  >11 publications with DBT and Connective Tissue
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