PDHX anticorps (Middle Region)

N° du produit ABIN5515028

Détail du produit anti-PDHX anticorps

Antigène: PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))

Épitope: Middle Region

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp PDHX est non-conjugé

Application: Western Blotting (WB)

Séquence: NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP

Purification: Affinity purified

Immunogène: The immunogen is a synthetic peptide directed towards the middle region of human PDHX

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Détails sur PDHX

Antigène: PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))

Autre désignation: PDHX (PDHX Produits)

Synonymes: anticorps DLDBP, anticorps E3BP, anticorps OPDX, anticorps PDX1, anticorps proX, anticorps AI481367, anticorps E3bp, anticorps Pdx1, anticorps Ac1164, anticorps zgc:66110, anticorps zgc:85977, anticorps MGC86218, anticorps PDHX, anticorps DDBDRAFT_0168431, anticorps DDBDRAFT_0230192, anticorps DDB_0168431, anticorps DDB_0230192, anticorps pyruvate dehydrogenase complex component X, anticorps pyruvate dehydrogenase complex, component X, anticorps pyruvate dehydrogenase complex component X L homeolog, anticorps E3-binding protein, anticorps PDHX, anticorps Pdhx, anticorps pdhx, anticorps pdhx.L, anticorps pdhX

Sujet: 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.

Alias Symbols: E3BP, OPDX, PDX1, proX, DLDBP

Protein Size: 501

ID gène: 8050

NCBI Accession: NM_001135024, NP_001128496

UniProt: O00330