Recombinant Human DBT Protein, GST-tagged

• Cat.No.: DBT-2376H
• Product Overview: Human DBT full-length ORF (BAG36008.1, 1 a.a. - 482 a.a.) recombinant protein with GST-tag at N-terminal.

Specification

• Species: Human
• Source: Wheat Germ
• Tag: GST
• Description: 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]
• Molecular Mass: 79.9 kDa
• AA Sequence: MAAVRMLRTWSRNAGKLICVRYFQTCGNVHVLKPNYVCFFGYPSFKYSHPHHFLKTTAALRGQVVQFKLSDIGEGIREVTVKEWYVKEGDTVSQFDSICEVQSDKASVTITSRYDGVIKKLYYNLDDIAYVGKPLVDIETEALKDSEEDVVETPAVSHDEHTHQEIKGRKTLATPAVRRLAMENNIKLSEVVGSGKDGRILKEDILNYLEKQTGAILPPSPKVEIMPPPPKPKDMTVPILVSKPPVFTGKDKTEPIKGFQKAMVKTMSAALKIPHFGYCDEIDLTELVKLREELKPIAFARGIKLSFMPFFLKAASLGLLQFPILNASVDENCQNITYKASHNIGIAMDTEQGLIVPNVKNVQICSIFDIATELNRLQKLGSVGQLSTTDLTGGTFTLSNIGSIGGTFAKPVIMPPEVAIGALGSIKAIPRFNQKGEVYKAQIMNVSWSADHRVIDGATMSRFSNLWKSYLENPAFMLLDLK
• Applications: Enzyme-linked Immunoabsorbent Assay
  Western Blot (Recombinant protein)
  Antibody Production
  Protein Array
• Notes: Best use within three months from the date of receipt of this protein.
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

Gene Information

• Gene Name: DBT dihydrolipoamide branched chain transacylase E2 [ Homo sapiens ]
• Official Symbol: DBT
• Synonyms: DBT; dihydrolipoamide branched chain transacylase E2; dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex; maple syrup urine disease); lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; BCKADE2; BCKAD-E2; BCKAD E2 subunit; dihydrolipoyl transacylase; branched chain acyltransferase, E2 component; dihydrolipoyllysine-residue (2-methylpropanoyl)transferase; branched-chain alpha-keto acid dehydrogenase complex component E2; E2 component of branched chain alpha-keto acid dehydrogenase complex; mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b); dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex; E2; E2B; BCATE2; MGC9061
• Gene ID: 1629
• mRNA Refseq: NM_001918
• Protein Refseq: NP_001909
• MIM: 248610
• UniProt ID: P11182