PDHE2 protein (GST tag) (His tag) (80-1378)
PDHE2 protein (GST tag) (His tag)
Overview
Overview
| Synonyms | Pyruvate Dehydrogenase Complex Component E2 protein, PDH-E2 protein, PDH E2 protein |
|---|---|
| Species | Human |
| Protein Type | Recombinant |
Specifications
| Residues | 168-313 aa |
|---|---|
| Expression System | E.coli |
| Grade & Purity | > 90% pure |
| Method of Purification | Purified using proprietary chromatographic techniques |
| Molecular Weight | 48 kDa |
| Tag/Conjugate | His-GST tag |
| Form & Buffer | Supplied in 50mM Na2PO4, pH 7.5, 400mM NaCl, 10% glycerol, 1mM EDTA and 2mM DTT |
Storage & Safety
| Storage | Store at 4 deg C for short term storage. Aliquot and store at -70 deg C for long term storage. Avoid repeated freeze/thaw cycles. |
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General Information
| Biological Significance | PDHE2 is the second component enzyme of pyruvate dehydrogenase complex (PDC). |
|---|---|
| Expression | Tissue and Cell Localization: PDHE2 is mainly expressed in the mitochondrial matrix. |
| Implications in Disease | Patients with primary biliary cirrhosis, a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum, show autoantibodies against the E2 component of pyruvate dehydrogenase complex. Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency); also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. |
| Post-Translational Modifications | Does not undergo any major post-translational modifications. |
| Database Links | Entrez Gene: 1737 Human, SwissProt: O00330 Human, Unigene: 335551 Human |
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