Pyruvate dehydrogenase protein X component, mitochondrial (PDHX) Recombinant Protein | PDHX recombinant protein

Recombinant Human Pyruvate dehydrogenase protein X component, mitochondrial (PDHX)

Cat. Num. AAA9018518

• Gene Names: PDHX; E3BP; OPDX; PDX1; proX; DLDBP
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Pyruvate dehydrogenase protein X component; mitochondrial (PDHX); Recombinant Human Pyruvate dehydrogenase protein X component; mitochondrial(Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex)(E3-binding protein)(E3BP)(Lipoyl-containing pyruvate dehydrogenase complex component X)(proX); PDHX recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 54-501aa, Full Length of Mature Protein
• Sequence: GDPIKILMPSLSPTMEEGNIVKWLKKEGEAVSAGDALCEIETDKAVVTLDASDDGILAKIVVEEGSKNIRLGSLIGLIVEEGEDWKHVEIPKDVGPPPPVSKPSEPRPSPEPQISIPVKKEHIPGTLRFRLSPAARNILEKHSLDASQGTATGPRGIFTKEDALKLVQLKQTGKITESRPTPAPTATPTAPSPLQATAGPSYPRPVIPPVSTPGQPNAVGTFTEIPASNIRRVIAKRLTESKSTVPHAYATADCDLGAVLKVRQDLVKDDIKVSVNDFIIKAAAVTLKQMPDVNVSWDGEGPKQLPFIDISVAVATDKGLLTPIIKDAAAKGIQEIADSVKALSKKARDGKLLPEEYQGGSFSISNLGMFGIDEFTAVINPPQACILAVGRFRPVLKLTEDEEGNAKLQQRQLITVTMSSDSRVVDDELATRFLKSFKANLENPIRLA

• Species: Homo sapiens (Human)
• Relevance: Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
• Reconstitution: We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20 degree C/-80 degree C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
• Preparation and Storage: Store at -20 degree C. For extended storage, store at -20 or -80 degree C.

Product Categories/Family for PDHX recombinant protein

Cancer

References

"Characterization of interactions of dihydrolipoamide dehydrogenase with its binding protein in the human pyruvate dehydrogenase complex."Park Y.H., Patel M.S.Biochem. Biophys. Res. Commun. 395:416-419(2010)

NCBI and Uniprot Product Information

• NCBI GI #: 12643417
• NCBI GeneID: 8050
• UniProt Accession #: O00330; O60221; Q96FV8; Q99783; B4DW62; D3DR11; E9PB14; E9PBP7
• Molecular Weight: 501
• NCBI Official Full Name: Pyruvate dehydrogenase protein X component, mitochondrial
• 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; pyruvate dehydrogenase complex, E3-binding protein subunit; lipoyl-containing pyruvate dehydrogenase complex component X; pyruvate dehydrogenase complex, lipoyl-containing component X; dihydrolipo
• UniProt Protein Name: Pyruvate dehydrogenase protein X component, mitochondrial
• Protein Family: Pyruvate dehydrogenase complex
• UniProt Gene Name: PDHX
• UniProt Synonym Gene Names: PDX1; E3BP
• UniProt Entry Name: ODPX_HUMAN

NCBI Description

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]

Uniprot Description

PDHX: a mitochondrial protein required for anchoring dihydrolipoamide dehydrogenase (E3) to the pyruvate dehydrogenase complexes (PDHC) of eukaryotes. This specific binding is essential for a PDHC function. Eukaryotic pyruvate dehydrogenase complexes are organized about a core consisting of the oligomeric dihydrolipoamide acetyl-transferase (E2), around which are arranged multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide dehydrogenase (E3), and protein X bound by non-covalent bonds. Belongs to the 2-oxoacid dehydrogenase family. Contains 1 lipoyl-binding domain.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 11p13

Cellular Component: mitochondrial matrix

Molecular Function: transferase activity, transferring acyl groups

Biological Process: cellular metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E3-binding Protein Deficiency