PDHX Antibody - middle region (ARP85052_P050)

Catalog No: ARP85052_P050

Host: Rabbit
Target Name: PDHX
Sample Tissue: Human PANC1 Whole Cell lysates
Antibody Dilution: 1ug/ml

Product Info

• Tested Species Reactivity: Human
• Predicted Species Reactivity: Human
• Product Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Clonality: Polyclonal
• Host: Rabbit
• Application: WB
• Reconstitution and Storage: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human PDHX
• Purification: Affinity purified
• Peptide Sequence: Synthetic peptide located within the following region: FRLSPAARNILEKHSLDASQGTATGPRGIFTKEDALKLVQLKQTGKITES
• Concentration: 0.5 mg/ml
• Blocking Peptide: For anti-PDHX (ARP85052_P050) antibody is Catalog # AAP85052

Target Info

• Gene Symbol: PDHX
• Gene Full Name: pyruvate dehydrogenase complex component X
• Alias Symbols: E3BP, OPDX, PDX1, proX, DLDBP, PDHXD
• NCBI Gene Id: 8050
• Protein Name: pyruvate dehydrogenase protein X component, mitochondrial
• Description of Target: 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.
• Uniprot ID: O00330
• Protein Accession #: NP_001128496.1
• Nucleotide Accession #: NM_001135024.1
• Protein Size (# AA): 501
• Molecular Weight: 55 kDa