購物車
幫助
021-54845833/15800441009
中文名稱：DLAT 抗原（重組蛋白）
英文名稱：DLAT Antigen (Recombinant Protein)
別 名： dihydrolipoamide S-acetyltransferase; DLTA; PDCE2; PDC-E2
儲 存： 冷凍（-20℃）
概 述
技術規格
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Full name: |
dihydrolipoamide S-acetyltransferase |
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Synonyms: |
DLTA; PDCE2; PDC-E2 |
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Swissprot: |
P10515 |
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Gene Accession: |
BC039084 |
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Purity: |
>85%, as determined by Coomassie blue stained SDS-PAGE |
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Expression system: |
Escherichia coli |
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Tags: |
His tag C-Terminus, GST tag N-Terminus |
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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. |
