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PDC-E2 Monoclonal Antibody

CAT: 0965-JOT-MCA1008-01Size: 20 µLDry Ice: NoHazardous: No
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CAT#:0965-JOT-MCA1008-01Size:20 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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 lact
CAS Number
9007-83-4
Synonyms
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
Host
Mouse
Reactivity
Human,Rb
Immunogen
Purified recombinant human PDC-E2 protein fragments expressed in E.coli.
Clonality
Monoclonal
Applications
WB
Stability
-20°C for one year
Concentration
1 mg/ml
Antibody Type
Primary antibody
Isotype
IgG

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