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Pyruvate Dehydrogenase E2 Recombinant Rabbit mAb (KU)

货号: BS45249

价格：￥ 1200

货期：现货（1-2个工作日）

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No. :BS45249

Product Name :Pyruvate Dehydrogenase E2 Recombinant Rabbit mAb (KU)

Swiss-Prot :P10515

Host :Rabbit

Reactivity :Human, Mouse, Rat

Applications :WB,ICC/IF,FC,IP

Application_all :WB: 1:1000-1:5000<br />ICC/IF: 1:50<br />FC: 1:20<br />IP: 1:20

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.[provided by RefSeq, Oct 2009]

Product :Store at -20°C. Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.

Purification&Purity :Affinity Purification

Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity :IgG

BiowMW :69 kDa

Note :For research use only, not for use in diagnostic procedure.

Alternative Name :E2; PBC; DLTA; PDCE2; PDC-E2

Immunogen :A synthetic peptide of human Pyruvate Dehydrogenase E2

Conjugate :Unconjugated

Modification :Unmodification

Western blot detection of Pyruvate Dehydrogenase E2 in CHO-K1,C6,Rat Brain,Ramos cell lysates using Pyruvate Dehydrogenase E2 antibody(1:1000 diluted).