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PDHX Produkte

(Pyruvate Dehydrogenase Complex, Component X (PDHX))

Kategorien

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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Ausgewählte PDHX Kategorien

PDHX Antikörper

High quality antibodies with extensive validation data.

PDHX ELISA Kits

Reliable ELISA kits for a wide range of species.

PDHX Proteine

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

Empfohlene PDHX Antikörper

Produkt
Reaktivität
Applikation
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Applikation WB, IHC, IP, ICC
Validierungen
  • (8)
Kat. Nr. ABIN7443305
Menge 100 μL
Datenblatt Datenblatt
Reaktivität Mouse
Applikation WB, IHC, IP, ICC
Validierungen
  • (7)
Kat. Nr. ABIN7443338
Menge 100 μL
Datenblatt Datenblatt
Reaktivität Human
Applikation WB, IHC, ELISA
Validierungen
  • (4)
Kat. Nr. ABIN7166903
Menge 100 μL
Datenblatt Datenblatt

Empfohlene PDHX ELISA Kits

Produkt
Reaktivität
Analytical Method
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Analytical Method Quantitative Sandwich ELISA
Validierungen
  • (1)
Kat. Nr. ABIN6959008
Menge 96 tests
Datenblatt Datenblatt
Reaktivität Human
Analytical Method Sandwich ELISA
Validierungen
Kat. Nr. ABIN423044
Menge 96 tests
Datenblatt Datenblatt

Empfohlene PDHX Proteine

Produkt
Reaktivität
Source
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Source Wheat germ
Validierungen
  • (1)
Kat. Nr. ABIN1314527
Menge 10 μg
Datenblatt Datenblatt
Reaktivität Human
Source Escherichia coli (E. coli)
Validierungen
  • (1)
Kat. Nr. ABIN7124721
Menge 50 μg
Datenblatt Datenblatt
Reaktivität Human
Source Escherichia coli (E. coli)
Validierungen
Kat. Nr. ABIN7416460
Menge 100 μg
Datenblatt Datenblatt

Neueste Publikationen zu unseren PDHX Produkten

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, 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." in: 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." in: Progress in nucleic acid research and molecular biology, Vol. 70, pp. 33-75, (2001) (PubMed).

Synonyme und alternative Namen zu 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

Bezeichner auf Proteinebene für 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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