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Mouse Monoclonal PDC-E2 antibody (STJ98527)
Supplier: St John’s Laboratory Ltd.
Recommended applications: WB
Recommended dilution: WB 1:1000-1:2000
Recommended protocols: check protocols
Click or hover above images to see image description for PDC-E2 Monoclonal Antibody.
Check alternative names for the antibodyExpand
DLAT antibody, DLTA antibody,||
SCBT cat No: To be updated
PDC-E2 Monoclonal Antibody
|Catalogue No.|| |
PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.
Purified recombinant human PDC-E2 protein fragments expressed in Ecoli
|Recommended dilution|| |
PDC-E2 Antibody was tube-contained. Purified in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.
PDC-E2 Antibody was purified using affinity purification.
-20 Celsius degree. Avoid repeated freeze/thaw cycles.
|Alternative antibody names|| |
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial antibody, 70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody, PBC antibody, Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody, M2 antigen complex 70 kDa subunit antibody, Pyruvate dehydrogenase complex component E2 antibody, PDC-E2 antibody, PDCE2 antibody
|Protein names|| |
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial , 70 kDa mitochondrial autoantigen of primary biliary cirrhosis , PBC , Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex , M2 antigen complex 70 kDa subunit , Pyruvate dehydrogenase complex component E2 , PDC-E2 , PDCE2
|Protein function|| |
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle. / Acetyl-CoA + enzyme N(6)-(dihydrolipoyl)lysine = CoA + enzyme N(6)-(S-acetyldihydrolipoyl)lysine. / (R)-lipoate
|Involvement in disease|| |
Note: Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients’ serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex.; Pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:245348]: Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent. . Note: The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the 2-oxoacid dehydrogenase family. / Contains 2 lipoyl-binding domains.
|Protein post-translational modifications|| |
Delipoylated at Lys-132 and Lys-259 by SIRT4, delipoylation decreases the PHD complex activity.
|Protein cellular localization|| |
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St John’s Laboratory Ltd.
|Product type|| |
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