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PDHX Produits

(Pyruvate Dehydrogenase Complex, Component X (PDHX))

Catégories

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].

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Catégories PDHX en vedette

PDHX Anticorps

High quality antibodies with extensive validation data.

PDHX Kits ELISA

Reliable ELISA kits for a wide range of species.

PDHX Protéines

Proteins for various applications incl. WB, ELISA, IF etc.

PDHX recommandé Anticorps

Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Application WB, IHC, IP, ICC
Validations
  • (8)
N° du produit ABIN7443305
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Mouse
Application WB, IHC, IP, ICC
Validations
  • (7)
N° du produit ABIN7443338
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Human
Application WB, IHC, ELISA
Validations
  • (4)
N° du produit ABIN7166903
Quantité 100 μL
Fiche technique Fiche technique

PDHX recommandé Kits ELISA

Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
N° du produit ABIN6959008
Quantité 96 tests
Fiche technique Fiche technique
Reactivity Human
Analytical Method Sandwich ELISA
Validations
N° du produit ABIN423044
Quantité 96 tests
Fiche technique Fiche technique

PDHX recommandé Protéines

Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1314527
Quantité 10 μg
Fiche technique Fiche technique
Reactivity Human
Source Escherichia coli (E. coli)
Validations
  • (1)
N° du produit ABIN7124721
Quantité 50 μg
Fiche technique Fiche technique
Reactivity Human
Source Escherichia coli (E. coli)
Validations
N° du produit ABIN7416460
Quantité 100 μg
Fiche technique Fiche technique

Dernières publications sur nos produits PDHX

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." dans: Journal of proteomics, Vol. 122, pp. 119-32, (2015) (PubMed).

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." dans: FEBS letters, Vol. 529, Issue 2-3, pp. 173-8, (2002) (PubMed).

Roche, Baker, Yan, Hiromasa, Gong, Peng, Dong, Turkan, Kasten: "Distinct regulatory properties of pyruvate dehydrogenase kinase and phosphatase isoforms." dans: Progress in nucleic acid research and molecular biology, Vol. 70, pp. 33-75, (2001) (PubMed).

Synonymes et noms alternatifs relatifs à PDHX

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

Protein level used designations for PDHX

  • 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)
  • pyruvate dehydrogenase complex, component X
  • E3-binding protein
  • E3BP
  • proX
  • pyruvate dehydrogenase protein X component
  • pyruvate dehydrogenase protein X component, mitochondrial-like
  • LOW QUALITY PROTEIN: pyruvate dehydrogenase protein X component, mitochondrial
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