Recombinant Human DLAT Protein, GST-tagged

• Cat.No.: DLAT-2670H
• Product Overview: Human DLAT full-length ORF ( NP_001922.2, 1 a.a. - 647 a.a.) recombinant protein with GST-tag at N-terminal.

Specification

• Description: This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009]
• Source: Wheat Germ
• Species: Human
• Tag: GST
• Molecular Mass: 95.4 kDa
• AA Sequence: MWRVCARRAQNVAPWAGLEARWTAL QEVPGTPRVTSRSGPAPARRNSVTT GYGGVRALCGWTPSSGATPRNRLLL QLLGSPGRRYYSLPPHQKVPLPSLS PTMQAGTIARWEKKEGDKINEGDLI AEVETDKATVGFESLEECYMAKILV AEGTRDVPIGAIICITVGKPEDIEA FKNYTLDSSAAPTPQAAPAPTPAAT ASPPTPSAQAPGSSYPPHMQVLLPA LSPTMTMGTVQRWEKKVGEKLSEGD LLAEIETDKATIGFEVQEEGYLAKI LVPEGTRDVPLGTPLCIIVEKEADI SAFADYRPTEVTDLKPQVPPPTPPP VAAVPPTPQPLAPTPSAPCPATPAG PKGRVFVSPLAKKLAVEKGIDLTQV KGTGPDGRITKKDIDSFVPSKVAPA PAAVVPPTGPGMAPVPTGVFTDIPI SNIRRVIAQRLMQSKQTIPHYYLSI DVNMGEVLLVRKELNKILEGRSKIS VNDFIIKASALACLKVPEANSSWMD TVIRQNHVVDVSVAVSTPAGLITPI VFNAHIKGVETIANDVVSLATKARE GKLQPHEFQGGTFTISNLGMFGIKN FSAIINPPQACILAIGASEDKLVPA DNEKGFDVASMMSVTLSCDHRVVDG AVGAQWLAEFRKYLEKPITMLL
• 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: DLAT dihydrolipoamide S-acetyltransferase [ Homo sapiens ]
• Official Symbol: DLAT
• Synonyms: DLAT; dihydrolipoamide S-acetyltransferase; DLTA; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; E2 component of pyruvate dehydrogenase complex; PDC E2; PBC; M2 antigen complex 70 kDa subunit; pyruvate dehydrogenase complex component E2; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial; PDCE2; PDC-E2
• Gene ID: 1737
• mRNA Refseq: NM_001931
• Protein Refseq: NP_001922
• MIM: 608770
• UniProt ID: P10515