丙酮酸脱氢酶
丙酮酸脱氢酶结构式
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常用名 | 丙酮酸脱氢酶 | 英文名 | Pyruvate Dehydrogenase |
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| CAS号 | 9014-20-4 | 分子量 | 297.305 | |
| 密度 | 1.3±0.1 g/cm3 | 沸点 | 508.4±50.0 °C at 760 mmHg | |
| 分子式 | C17H15NO4 | 熔点 | N/A | |
| MSDS | 美版 | 闪点 | 261.3±30.1 °C |
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Alternating sites reactivity is a common feature of thiamin diphosphate-dependent enzymes as evidenced by isothermal titration calorimetry studies of substrate binding.
Biochemistry 52(15) , 2505-7, (2013) Thiamin diphosphate (ThDP)-dependent enzymes play vital roles in cellular metabolism in all kingdoms of life. In previous kinetic and structural studies, a communication between the active centers in terms of a negative cooperativity had been suggested for so... |
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Enhanced glucose metabolism is preserved in cultured primary myotubes from obese donors in response to exercise training.
J. Clin. Endocrinol. Metab. 98(9) , 3739-47, (2013) It was suggested that human cultured primary myotubes retain the metabolic characteristics of their donor in vitro.The aim of the present study was to investigate whether the metabolic responses to endurance training are also conserved in culture.Middle-aged ... |
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Intermittent-relapsing pyruvate dehydrogenase complex deficiency: a case with clinical, biochemical, and neuroradiological reversibility.
Dev. Med. Child Neurol. 54(5) , 472-6, (2012) Pyruvate dehydrogenase complex (PDHC) deficiency causes encephalomyopathies, of which there are four major categories: (1) neonatal encephalopathy with lactic acidosis; (2) an early infantile form, which (3) at times resembles Leigh syndrome; and (4) a later-... |
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Development of multilocus putatively neutral DNA markers in the X-chromosome for population genetic studies in humans.
Ann. Hum. Biol. 39(4) , 281-9, (2012) It has now been well documented that the type (coding, non-coding) and location (nuclear, mitochondrial etc.) of genetic markers heavily influence evolutionary inferences; realistic assumptions can be drawn if multiple putatively neutral DNA fragments spread ... |
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Spectrum of neurological and survival outcomes in pyruvate dehydrogenase complex (PDC) deficiency: lack of correlation with genotype.
Mol. Genet. Metab. 107(3) , 394-402, (2012) Pyruvate dehydrogenase complex (PDC) deficiency is a relatively common mitochondrial disorder that primarily presents with neurological manifestations and lactic acidemia. We analyzed the clinical outcomes and neurological features of 59 consented symptomatic... |
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Unilateral periventricular leukomalacia in association with pyruvate dehydrogenase deficiency.
Dev. Med. Child Neurol. 54(5) , 469-71, (2012) Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. A deficiency of PDH E1 alpha, a subunit of the PDH complex, is a prominent cause of congenital lactic acidosis. We... |
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Increased superoxide accumulation in pyruvate dehydrogenase complex deficient fibroblasts.
Mol. Genet. Metab. 104(3) , 255-60, (2011) The pyruvate dehydrogenase complex (PDC) oxidizes pyruvate to acetyl CoA and is critically important in maintaining normal cellular energy homeostasis. Loss-of-function mutations in PDC give rise to congenital lactic acidosis and to progressive cellular energ... |
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Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene.
Brain Dev. 34(2) , 87-91, (2012) Leigh syndrome (LS) is a progressive untreatable degenerating mitochondrial disorder caused by either mitochondrial or nuclear DNA mutations. A patient was a second child of unconsanguineous parents. On the third day of birth, he was transferred to neonatal i... |
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Growth factor withdrawal in combination with sodium butyrate addition extends culture longevity and enhances antibody production in CHO cells.
J. Biotechnol. 155(2) , 225-31, (2011) The effect of growth factor (GF) and sodium butyrate (NaBu) on Chinese hamster ovary (CHO) cell growth, cell viability and antibody production was investigated using shaking flasks in GF-containing and GF-deficient medium containing 0, 1 and 3mM NaBu. The wit... |
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Demethylation of the coding region triggers the activation of the human testis-specific PDHA2 gene in somatic tissues.
PLoS ONE 7(6) , e38076, (2012) Human PDHA2 is a testis-specific gene that codes for the E(1)α subunit of Pyruvate Dehydrogenase Complex (PDC), a crucial enzyme system in cell energy metabolism. Since activation of the PDHA2 gene in somatic cells could be a new therapeutic approach for PDC ... |