DESCRIPTION

• Note: Single siRNA duplex (10nmol) can be ordered.
• Purity: HPLC purified
• Refseq: NM_001135024, NM_001166158, NM_003477
• Status: In Stock*
• Summary: The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor 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 eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
• Category: RNAi
• Synonyms: DLDBP; E3BP; OPDX; PDHXD; PDX1; proX
• Sequences: Available with shipment
• Stability: One year from date of shipment when stored at -20°C.
• Components: PDHX (Human) - 3 unique 27mer siRNA duplexes - 2 nmol each (Locus ID 8050)Included - SR30004, Trilencer-27 Universal Scrambled Negative Control siRNA Duplex - 2 nmolIncluded - SR30005, RNAse free siRNA Duplex Resuspension Buffer - 2 ml Need an individual siRNA?
• Uniprot Id: O00330
• Availability: In Stock
• Quality Control: Tested by ESI-MS
• Performance Guaranteed: OriGene guarantees that at least two of the three Dicer-Substrate duplexes in the kit will provide at least 70% or more knockdown of the target mRNA when used at 10 nM concentration by quantitative RT-PCR when the TYE-563 fluorescent transfection control duplex (cat# SR30002) indicates that >90% of the cells have been transfected and the HPRT positive control (cat# SR30003) provides 90% knockdown efficiency. For non-conforming siRNA, requests for replacement product must be made within ninety (90) days from the date of delivery of the siRNA kit. To arrange for a free replacement with newly designed duplexes, please contact Technical Services at techsupport@origene.com. Please provide your data indicating the transfection efficiency and measurement of gene expression knockdown compared to the scrambled siRNA control (quantitative RT-PCR data required).
• Number of Transfections: Approximately 330 transfections/2nmol in 24-well plate under optimized conditions (final conc. 10 nM).