Anti-Human/Mouse/Rat DLAT Polyclonal Antibody

DLAT多克隆抗体

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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基本信息

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货号 PA07479
规格 100μl
宿主 Rabbit
免疫原 Fusion protein of human DLAT
特异性
亚型 IgG
反应性 Human,Mouse,Rat
克隆
Uniprot P10515
浓度 0.6 mg/mL
推荐稀释度 WB 1:500-2000, IHC 1:30-150, ELISA 1:2000-5000
配方
应用范围 WB,IHC,ELISA
别名 70 kDa mitochondrial autoantigen of primary biliary cirrhosis,Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex,Dihydrolipoamide S Acetyltransferase,Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex,dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial,DLAT,DLTA,E2,E2 component of pyruvate dehydrogenase complex,M2 antigen complex 70 kDa subunit,mitochondrial,ODP2,PBC,PDC E2,PDC-E2,PDCE2,Pyruvate dehydrogenase complex component E2,Pyruvate dehydrogenase complex E2 subunit
保存条件 冻干产品: 2 - 8°C保存5年; 液体: -20°C保存2年.
注意 仅用于科学研究, 不能用于疾病诊断.

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