丙酮酸脱氢酶E2抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 丙酮酸脱氢酶E2抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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 丙酮酸脱氢酶E2抗体(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]

丙酮酸脱氢酶E2抗体Also known as:

Pyruvate Dehydrogenase E2; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; anti DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide; Dihydrolipoamide S Acetyltransferase; Dihydrolipoamide S-acetyltransferase (E2 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;丙酮酸脱氢酶E2抗体 E2; E2 component of pyruvate dehydrogenase complex; EC 2.3.1.12; M2 antigen complex 70 kDa subunit; M2 Antigen Complex 70kD Subunit; mitochondrial; ODP2_HUMAN; PBC; PDC E2; PDC-E2; PDCE2; Pyruvate dehydrogenase complex component E2; Pyruvate dehydrogenase complex E2 subunit; S acetyltransferase component of pyruvate dehydrogenase complex.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, Guinea Pig, Cat, ,Danio rerio.

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Immunogen: KLH conjugated synthetic peptide derived from human PDCE2.

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丙酮酸脱氢酶E2抗体Predicted Molecular Weight: 63kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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