PDHX antibody (Middle Region)

Catalog No. ABIN5515028

Product Details anti-PDHX Antibody

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

Binding Specificity: Middle Region

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This PDHX antibody is un-conjugated

Application: Western Blotting (WB)

Sequence: NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP

Purification: Affinity purified

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

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

Format: Liquid

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

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: 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.

Target Details for PDHX

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

Alternative Name: PDHX (PDHX Products)

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

Background: 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

Gene ID: 8050

NCBI Accession: NM_001135024, NP_001128496

UniProt: O00330