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
Insect Cells
Validations
- (1)
N° du produit
ABIN3085027
Quantité
1 mg
Fiche technique
Fiche technique
Dernières publications sur nos produits PDHX
: "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).: "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).
: "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:85977Protein 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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