中文名称:DLAT 抗原(重组蛋白)
英文名称:DLAT Antigen (Recombinant Protein)
别 名:dihydrolipoamide S-acetyltransferase; DLTA; PDCE2; PDC-E2
储 存:冷冻(-20℃)
相关类别:抗原
概 述Fusion protein corresponding to C terminal 200 amino acids of human DLAT技术规格
Full name:
dihydrolipoamide S-acetyltransferase
Synonyms:
DLTA; PDCE2; PDC-E2
Swissprot:
P10515
Gene Accession:
BC039084
Purity:
>85%, as determined by Coomassie blue stained SDS-PAGE
Expression system:
Escherichia coli
Tags:
His tag C-Terminus, GST tag N-Terminus
Background:
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.
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