PDHX / PDX1 (54-501, His-tag) Human Protein

CAT#: AR51757PU-S

PDHX / PDX1 (54-501, His-tag) human recombinant protein, 20 µg

Product Images

Specifications

Product Data

• Species: Human
• Expression Host: E. coli
• Expression cDNA Clone or AA Sequence:
  Protein Sequence

  MGSSHHHHHH SSGLVPRGSH MGSGDPIKIL MPSLSPTMEE GNIVKWLKKE GEAVSAGDAL CEIETDKAVV TLDASDDGIL AKIVVEEGSK NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP VKKEHIPGTL RFRLSPAARN ILEKHSLDAS QGTATGPRGI FTKEDALKLV QLKQTGKITE SRPTPAPTAT PTAPSPLQAT AGPSYPRPVI PPVSTPGQPN AVGTFTEIPA SNIRRVIAKR LTESKSTVPH AYATADCDLG AVLKVRQDLV KDDIKVSVND FIIKAAAVTL KQMPDVNVSW DGEGPKQLPF IDISVAVATD KGLLTPIIKD AAAKGIQEIA DSVKALSKKA RDGKLLPEEY QGGSFSISNL GMFGIDEFTA VINPPQACIL AVGRFRPVLK LTEDEEGNAK LQQRQLITVT MSSDSRVVDD ELATRFLKSF KANLENPIRL A
• Tag: His-tag
• Predicted MW: 50.4 kDa
• Concentration: lot specific
• Purity: >85% by SDS - PAGE
• Buffer: Presentation State: Purified
  State: Liquid purified protein
  Buffer System: Phosphate buffer saline (pH 7.4) containing 20% glycerol, 1mM DTT.
• Preparation: Liquid purified protein
• Protein Description: Recombinant human PDHX protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
• Storage: Store undiluted at 2-8°C for one week or (in aliquots) at -20°C to -80°C for longer.
  Avoid repeated freezing and thawing.
  
• Stability: Shelf life: one year from despatch.

Reference Data

• RefSeq: NP_001128496
• Locus ID: 8050
• UniProt ID: O00330
• Cytogenetics: 11p13
• Synonyms: DLDBP; E3BP; OPDX; PDHXD; PDX1; proX
• 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]