Rabbit anti-Human PDHX Polyclonal Antibody | anti-PDHX antibody

PDHX antibody

Cat. Num. AAA306511

• Gene Names: PDHX; E3BP; OPDX; PDX1; proX; DLDBP
• Reactivity: Human
• Applications: Immunofluorescence, Western Blot
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: PDHX, Antibody; PDHX antibody; E3BP; OPDX; PDX1; proX; DLDBP; anti-PDHX antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total PDHX antibody.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 486

• Applicable Applications for anti-PDHX antibody: IF (Immunofluorescence), WB (Western Blot)
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human PDHX.
• Target Name: PDHX
• Preparation and Storage: Store at -20 degree C

IF (Immunofluorescence)

(Immunofluorescence analysis of U2OS cell using PDHX antibody. Blue: DAPI for nuclear staining.)

WB (Western Blot)

(Western blot analysis of extracts of various cell lines, using PDHX antibody.)

Related Product Information for anti-PDHX antibody

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.

Product Categories/Family for anti-PDHX antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 203098816
• NCBI GeneID: 8050
• NCBI Accession #: NP_001128496.1
• NCBI GenBank Nucleotide #: NM_001135024.1
• UniProt Accession #: O00330; O60221; Q96FV8; Q99783; B4DW62; D3DR11; E9PB14; E9PBP7
• Molecular Weight: 51,460 Da
• NCBI Official Full Name: pyruvate dehydrogenase protein X component, mitochondrial isoform 2
• NCBI Official Synonym Full Names: pyruvate dehydrogenase complex, component X
• NCBI Official Symbol: PDHX
• NCBI Official Synonym Symbols: E3BP; OPDX; PDX1; proX; DLDBP
• NCBI Protein Information: pyruvate dehydrogenase protein X component, mitochondrial
• UniProt Protein Name: Pyruvate dehydrogenase protein X component, mitochondrial
• UniProt Gene Name: PDHX
• UniProt Synonym Gene Names: PDX1; E3BP
• UniProt Entry Name: ODPX_HUMAN