PDHX 产品
(Pyruvate Dehydrogenase Complex, Component X (PDHX))
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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. 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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Featured PDHX Categories
PDHX 抗体
High quality antibodies with extensive validation data.
PDHX ELISA试剂盒
Reliable ELISA kits for a wide range of species.
PDHX 蛋白
Proteins for various applications incl. WB, ELISA, IF etc.
Recommended PDHX 抗体
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Cat. No.
ABIN7443305
Quantity
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Mouse
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Cat. No.
ABIN7443338
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Cat. No.
ABIN7166903
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Recommended PDHX ELISA试剂盒
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ABIN6959008
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Recommended PDHX 蛋白
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Wheat germ
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Cat. No.
ABIN1314527
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10 μg
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Escherichia coli (E. coli)
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Cat. No.
ABIN7124721
Quantity
50 μg
Datasheet
Datasheet
Reactivity
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Source
Escherichia coli (E. coli)
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Cat. No.
ABIN7416460
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Latest Publications for our PDHX Products
: "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).: "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).
: "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).
Synonyms and alternative names related to 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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