GTX47093
DLAT antibody, N-term
Eine Lieferung in diese Region ist nicht möglich.
- SKU:
- GTX47093
- Zusätzliche Namen:
- dihydrolipoamide S-acetyltransferase , DLTA , E2 , PDC-E2 , PDCE2
- Anwendung:
- WB, IHC-P
- Konzentration:
- 0.5-1 mg/ml
- Physischer Zustand:
- Liquid
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Protein A Purified
- Lagerbedingungen:
- -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
- Hersteller:
- Genetex
- Host:
- Rabbit
- Reaktivitäten:
- Human
- Buffer:
- PBS, 2% Sucrose, 0.09% Sodium azide.
- Immunogen:
- A synthetic peptide corresponding to a N-terminal region of Human DLAT
- Uniprot:
- P10515
- Synonyme:
- 70 kDa mitochondrial autoantigen of primary biliary cirrhosis;dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial;DLTA;E2;E2 component of pyruvate dehydrogenase complex;M2 antigen complex 70 kDa subunit;PBC;PDC-E2;PDCE2;pyruvate dehydrogenase complex component E2
- Weitere Details:
- 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]
- Versandbedingungen:
- Blue Ice