排序方式: 共有3条查询结果,搜索用时 0 毫秒
1
1.
Mezzomo Nathana Jamille Becker Borin Diego Ianiski Francine Dotto Fontana Barbara Diehl de Franceschi Itiane Bolzan Juliane Garcez Renata Grings Mateus Parmeggiani Belisa da Silva Fernandes Liana de Almeida Vaucher Rodrigo Leipnitz Guilhian Duval Wannmacher Clovis Milton Cielo Rech Virginia 《Molecular biology reports》2019,46(6):5897-5908
Molecular Biology Reports - Phenylketonuria (PKU) is a metabolic disorder accumulating phenylalanine (Phe) and its metabolites in plasma and tissues of the patients. Regardless of the mechanisms,... 相似文献
2.
Itiane Diehl de Franceschi Elenara Rieger Alessandra Pinto Vargas Denise Bertin Rojas Aline Guimarães Campos Virginia Cielo Rech Luciane Rosa Feksa Clóvis Milton Duval Wannmacher 《Neurochemical research》2013,38(3):632-643
Maple Syrup Urine Disease is an inborn error of metabolism caused by severe deficiency in the activity of branched-chain α-keto acid dehydrogenase complex. Neurological disorder is common in patients with maple syrup urine disease. Although leucine is considered the main toxic metabolite, the mechanisms underlying the neuropathology of brain injury are poorly understood. In the present study, we evaluated the possible preventive effect of the co-administration of creatine plus pyruvate on the effects elicited by leucine administration to female Wistar rats during pregnancy and lactation on some oxidative stress parameters as well as the activities of some enzymes involved in the phosphoryltransfer network in the brain cortex and hippocampus of the offspring at 21 days of age. Leucine administration induced oxidative stress and altered the activities of pyruvate kinase, adenylate kinase, mitochondrial and cytosolic creatine kinase. Co-administration of creatine plus pyruvate was partially effective in the prevention of some alterations provoked by leucine administration on the oxidative stress but not in the enzymes of phosphoryltransfer network. These results suggest that non-treated maternal hyperleucinemia may be toxic to the brain of the offspring. 相似文献
3.
Vanessa Trindade Bortoluzzi Itiane Diehl de Franceschi Elenara Rieger Clóvis Milton Duval Wannmacher 《Neurochemical research》2014,39(8):1594-1602
Phenylketonuria (PKU) is the most frequent inborn error of metabolism. It is caused by deficiency in the activity of phenylalanine hydroxylase, leading to accumulation of phenylalanine and its metabolites. Untreated maternal PKU or hyperphenylalaninemia may result in nonphenylketonuric offspring with low birth weight and neonatal sequelae, especially microcephaly and intellectual disability. The mechanisms underlying the neuropathology of brain injury in maternal PKU syndrome are poorly understood. In the present study, we evaluated the possible preventive effect of the co-administration of creatine plus pyruvate on the effects elicited by phenylalanine administration to female Wistar rats during pregnancy and lactation on some enzymes involved in the phosphoryltransfer network in the brain cortex and hippocampus of the offspring at 21 days of age. Phenylalanine administration provoked diminution of body, brain cortex an hippocampus weight and decrease of adenylate kinase, mitochondrial and cytosolic creatine kinase activities. Co-administration of creatine plus pyruvate was effective in the prevention of those alterations provoked by phenylalanine, suggesting that altered energy metabolism may be important in the pathophysiology of maternal PKU. If these alterations also occur in maternal PKU, it is possible that pyruvate and creatine supplementation to the phenylalanine-restricted diet might be beneficial to phenylketonuric mothers. 相似文献
1