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Dihydrolipoamide Branched Chain Transacylase E2 (DBT)

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BCATE2; 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Dihydrolipoamide branched chain transacylase; Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase

Dihydrolipoamide Branched Chain Transacylase E2 (DBT)
CCC J294
HGNC 2698
MGI 105386
PubMed 22046030
RGD 68403
UniProt P11182
Wiki 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.The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase. The DBT gene encodes the E2 component.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dihydrolipoamide Branched Chain Transacylase E2 (DBT) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dihydrolipoamide Branched Chain Transacylase E2 (DBT) ELISA Kit Customized Service Offer
  1. "The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain alpha-keto acid dehydrogenase complex."Biochim. Biophys. Acta 1132:319-321(1992) [PubMed] [Europe PMC] [Abstract]
  2. "Nucleotide sequence of a cDNA for branched chain acyltransferase with analysis of the deduced protein structure."J. Biol. Chem. 263:6165-6168(1988) [PubMed] [Europe PMC] [Abstract]
  3. "Construction and nucleotide sequence of a cDNA encoding the full-length preprotein for human branched chain acyltransferase."J. Biol. Chem. 264:7742-7746(1989) [PubMed] [Europe PMC] [Abstract]
  4. "Complete primary structure of the transacylase (E2b) subunit of the human branched chain alpha-keto acid dehydrogenase complex."Biochem. Biophys. Res. Commun. 161:1035-1041(1989) [PubMed] [Europe PMC] [Abstract]
  5. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  6. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
  7. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  8. "Conservation of primary structure in the lipoyl-bearing and dihydrolipoyl dehydrogenase binding domains of mammalian branched-chain alpha-keto acid dehydrogenase complex: molecular cloning of human and bovine transacylase (E2) cDNAs."Biochemistry 27:1972-1981(1988) [PubMed] [Europe PMC] [Abstract]
  9. "Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene."J. Biol. Chem. 267:24090-24096(1992) [PubMed] [Europe PMC] [Abstract]
  10. "Differential processing of human and rat E1 alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence."Biochim. Biophys. Acta 1201:125-128(1994) [PubMed] [Europe PMC] [Abstract]
  11. "Reactivity of primary biliary cirrhosis sera with a human fetal liver cDNA clone of branched-chain alpha-keto acid dehydrogenase dihydrolipoamide acyltransferase, the 52 kD mitochondrial autoantigen."Hepatology 9:63-68(1989) [PubMed] [Europe PMC] [Abstract]
  12. "Autoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope."Hepatology 22:505-513(1995) [PubMed] [Europe PMC] [Abstract]
  13. "Comparative studies of antimitochondrial autoantibodies in sera and bile in primary biliary cirrhosis."Hepatology 25:1085-1089(1997) [PubMed] [Europe PMC] [Abstract]
  14. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
  15. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  16. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  17. "Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex."J. Biol. Chem. 277:15865-15873(2002) [PubMed] [Europe PMC] [Abstract]
  18. "Solution structure of the E3-binding domain of dihydrolipoamide branched chain transacylase."Submitted (NOV-2005) to the PDB data bank
  19. "A 17-bp insertion and a Phe215-->Cys missense mutation in the dihydrolipoyl transacylase (E2) mRNA from a thiamine-responsive maple syrup urine disease patient WG-34."Biochem. Biophys. Res. Commun. 174:804-809(1991) [PubMed] [Europe PMC] [Abstract]
  20. "Molecular basis of intermittent maple syrup urine disease: novel mutations in the E2 gene of the branched-chain alpha-keto acid dehydrogenase complex."J. Hum. Genet. 43:91-100(1998) [PubMed] [Europe PMC] [Abstract]