- 产品信息
商品描述
靶点-Target
PDC-E2
反应性
Human,Rabbit
应用
WB
宿主物种-Host Species
Mouse
修饰-Modification
Unmodified
MW(Calculated)
69kD
推荐稀释比
WB 1:1000-1:2000;Not yet tested in other applications.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
Affinity purification
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
克隆性
Monoclonal
免疫原
Purified recombinant human PDC-E2 protein fragments expressed in E.coli.
特异性
PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.
基因名称
DLAT
蛋白名称
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
别名
DLAT;DLTA;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;mitochondrial;70 kDa mitochondrial autoantigen of primary biliary cirrhosis;PBC;Dihydrolipoamide acetyltransferase component of pyruva
Organism-1
Human
基因ID-1
1737
SwissProt-1
P10515
Organism-2
Mouse
SwissProt-2
Q8BMF4
Organism-3
Rat
SwissProt-3
P08461
背景
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 lact
细胞定位
Mitochondrion matrix.





