Pyruvate Dehydrogenase E2 mouse mAb

CAT: 1027-RA11913-01Size: 50 µLDry Ice: NoHazardous: No
CAT#:1027-RA11913-01Size:50 µL
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AVAILABILITY: InStock
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Background
Dihydrolipoamide S-acetyltransferase (DLAT) Homo sapiens 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
Product Name Alternative
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; DLAT; DLTA; 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.
Gene ID
1737
Swiss Prot
P10515
Cross Reactivity
Human; Mouse
Clonality
Monoclonal
Source
Mouse
Applications
WB; ICC; IP
Dilution
Wb 1:1000 icc 1:300
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Molecular Weight
69
Storage Conditions
-20°C/1 year
Protein Weight
69

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