Pyruvate Dehydrogenase E2 Rabbit pAb
Product Name :
Pyruvate Dehydrogenase E2 Rabbit pAb
Cat.No.:
db20414
Synonyms :
E2; PBC; DLTA; PDCE2; PDC-E2
Reactivity :
Human, Mouse, Rat
Host species :
Rabbit
Conjugation/Formulation：Un-conjugated
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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 lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009]
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Immunogen
A synthetic peptide of human Pyruvate Dehydrogenase E2
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Gene ID
1737
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Swiss Prot
P10515
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Synonyms
E2; PBC; DLTA; PDCE2; PDC-E2
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Reactivity
Human, Mouse, Rat
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Application
WB, ICC/IF, FC, IP
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Recommended dilution
WB: 1:1000
ICC/IF: 1:20
FC: 1:20
IP: 1:20
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Calculated MW
69 kDa
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Observed MW
69 kDa
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Host species
Rabbit
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Clonality
Polyclonal
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Isotype
IgG
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Purity
Affinity Purification
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Conjugation
Un-conjugated
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Storage/Stability
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.
订购信息
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Package
Price (RMB)
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20μL
598
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50μL
998
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100μL
1798
- 货期：现货
For research use only. Not intended for diagnostic.
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