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The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. 再加上，我们可以发Pyruvate Dehydrogenase Complex, Component X 蛋白 (7) 和 Pyruvate Dehydrogenase Complex, Component X 试剂盒 (1)和数多这个蛋白质的别的产品。
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Human Polyclonal PDHX Primary Antibody for EIA, WB - ABIN4620452
Ling, McEachern, Seyda, MacKay, Scherer, Bratinova, Beatty, Giovannucci-Uzielli, Robinson: Detection of a homozygous four base pair deletion in the protein X gene in a case of pyruvate dehydrogenase complex deficiency. in Human molecular genetics 1998
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Human Polyclonal PDHX Primary Antibody for EIA, IHC (p) - ABIN4620453
Murray, Gilkerson, Capaldi: Quantitative proteomics: the copy number of pyruvate dehydrogenase is more than 10(2)-fold lower than that of complex III in human mitochondria. in FEBS letters 2002
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Human Polyclonal PDHX Primary Antibody for IF, IHC (p) - ABIN388774
Aral, Benelli, Ait-Ghezala, Amessou, Fouque, Maunoury, Créau, Kamoun, Marsac: Mutations in PDX1, the human lipoyl-containing component X of the pyruvate dehydrogenase-complex gene on chromosome 11p1, in congenital lactic acidosis. in American journal of human genetics 1998
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Human Polyclonal PDHX Primary Antibody for WB - ABIN388773
Harris, Bowker-Kinley, Wu, Jeng, Popov: Dihydrolipoamide dehydrogenase-binding protein of the human pyruvate dehydrogenase complex. DNA-derived amino acid sequence, expression, and reconstitution of the pyruvate dehydrogenase complex. in The Journal of biological chemistry 1997
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Human Polyclonal PDHX Primary Antibody for IHC, ELISA - ABIN185389
Roche, Baker, Yan, Hiromasa, Gong, Peng, Dong, Turkan, Kasten: Distinct regulatory properties of pyruvate dehydrogenase kinase and phosphatase isoforms. in Progress in nucleic acid research and molecular biology 2001
Human Polyclonal PDHX Primary Antibody for IHC (p), IHC - ABIN250349
Schild, Ruhs, Beiter, Zügel, Hudemann, Reimer, Krumholz-Wagner, Wagner, Keller, Eder, Krüger, Krüger, Braun, Nieß, Steinacker, Mooren: Basal and exercise induced label-free quantitative protein profiling of m. vastus lateralis in trained and untrained individuals. in Journal of proteomics 2015
We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis.
MiR (显示 MLXIP 抗体)-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX.
New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase (显示 PDP 抗体) deficiency.
genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 (显示 CD44 抗体) was established.
determination that PDH (显示 PDP 抗体) and complex III exist at a steady-state ratio of 1:100, 1:128 and 1:202 in HeLa cell extracts, fibroblast mitochondria and heart tissue mitochondria, respectively
model of the pyruvate dehydrogenase (显示 PDP 抗体) complex formed by E2 and E2 plus the E3-binding protein and binding of the E1 and E3 components
specificity of pairing for human E3BP with E3 from its subcomplex structure to be most likely due to conformational rigidity of the binding fragment of the E3-binding domain of E3BP and its exquisite amino acid match with the E3 target interface
A cluster of disease-causing E3 mutations located near the center of the E3BD/E3 interface prevents the efficient recruitment of these E3 variants by E3BP into the PDC (显示 PNKD 抗体), leading to the dysfunction of the PDC (显示 PNKD 抗体) catalytic machine.
These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene.
Despite the presence of antibodies reactive with PDC (显示 PNKD 抗体)-E3BP in the majority of primary biliary cirrhosis (PBC (显示 DLAT 抗体)) patients this self-protein is not a dominant T-cell autoantigen in PBC (显示 DLAT 抗体).
T1a/podoplanin (显示 PDPN 抗体) is first expressed in prox 1 (显示 PROX1 抗体)-positive lymphatic progenitor cells, with predominant localization in the luminal plasma membrane of lymphatic endothelial cells during later development.
In islet cells both fat and glucose regulate PDK (显示 PDK2 抗体) gene and protein expression and indicate that hyperglycemia and hyperlipidemia contribute to the decline in diabetic islet PDH (显示 PDP 抗体) activity by increasing mRNA and protein expression of PDK (显示 PDK2 抗体).
This study showed that the recombinant and bovine E2/E3BP cores bind E3s with a 2:1 stoichiometry, and propose that mammalian PDC (显示 PDC 抗体) comprises a heterogeneous population of assemblies incorporating a network of E3 cross-bridges above the core surface.
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.
pyruvate dehydrogenase complex, component X
, pyruvate dehydrogenase protein X component, mitochondrial-like
, dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex
, lipoyl-containing pyruvate dehydrogenase complex component X
, pyruvate dehydrogenase complex, E3-binding protein subunit
, pyruvate dehydrogenase complex, lipoyl-containing component X
, pyruvate dehydrogenase protein X component, mitochondrial
, dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex (E3-binding protein) (proX)
, E3-binding protein
, pyruvate dehydrogenase protein X component