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1.
Grings Mateus Wajner Moacir Leipnitz Guilhian 《Cellular and molecular neurobiology》2022,42(3):565-575
Cellular and Molecular Neurobiology - Ethylmalonic encephalopathy (EE) is a severe intoxication disorder caused by mutations in the ETHE1 gene that encodes a mitochondrial sulfur dioxygenase... 相似文献
2.
Bruna M. Schweinberger André F. Rodrigues Elias Turcatel Paula Pierozan Leticia F. Pettenuzzo Mateus Grings Giselli Scaini Mariana M. Parisi Guilhian Leipnitz Emilio L. Streck Florencia M. Barbé-Tuana Angela T. S. Wyse 《Molecular neurobiology》2018,55(2):980-988
In the current study, we verified the effects of maternal hypermethioninemia on the number of neurons, apoptosis, nerve growth factor, and brain-derived neurotrophic factor levels, energy metabolism parameters (succinate dehydrogenase, complex II, and cytochrome c oxidase), expression and immunocontent of Na+,K+-ATPase, edema formation, inflammatory markers (tumor necrosis factor-alpha and interleukin-6), and mitochondrial hydrogen peroxide levels in the encephalon from the offspring. Pregnant Wistar rats were divided into two groups: the first one received saline (control) and the second group received 2.68 μmol methionine/g body weight by subcutaneous injections twice a day during gestation (approximately 21 days). After parturition, pups were killed at the 21st day of life for removal of encephalon. Neuronal staining (anti-NeuN) revealed a reduction in number of neurons, which was associated to decreased nerve growth factor and brain-derived neurotrophic factor levels. Maternal hypermethioninemia also reduced succinate dehydrogenase and complex II activities and increased expression and immunocontent of Na+,K+-ATPase alpha subunits. These results indicate that maternal hypermethioninemia may be a predisposing factor for damage to the brain during the intrauterine life. 相似文献
3.
Mateus Struecker da Rosa Bianca Seminotti César Augusto João Ribeiro Belisa Parmeggiani Mateus Grings Moacir Wajner 《Free radical research》2016,50(9):997-1010
3-Hydroxy-3-methylglutaryl-coenzyme A lyase (HL) deficiency is characterized by tissue accumulation of 3-hydroxy-3-methylglutaric (HMG), and 3-methylglutaric (MGA) acids. Affected patients present cardiomyopathy, whose pathomechanisms are not yet established. We investigated the effects of HMG and MGA on energy and redox homeostasis in rat heart using in vivo and in vitro models. In vivo experiments showed that intraperitoneal administration of HMG and MGA decreased the activities of the respiratory chain complex II and creatine kinase (CK), whereas HMG also decreased the activity of complex II–III. Furthermore, HMG and MGA injection increased reactive species production and carbonyl formation, and decreased glutathione concentrations. Regarding the enzymatic antioxidant defenses, HMG and MGA increased glutathione peroxidase (GPx) and glutathione reductase (GR) activities, while only MGA diminished the activities of superoxide dismutase (SOD) and catalase, as well as the protein content of SOD1. Pre-treatment with melatonin (MEL) prevented MGA-induced decrease of CK activity and SOD1 levels. In vitro results demonstrated that HMG and MGA increased reactive species formation, induced lipid peroxidation and decreased glutathione. We also verified that reactive species overproduction and glutathione decrease provoked by HMG and MGA were abrogated by MEL and lipoic acid (LA), while only MEL prevented HMG- and MGA-induced lipoperoxidation. Allopurinol (ALP) also prevented reactive species overproduction caused by both metabolites. Our data provide solid evidence that bioenergetics dysfunction and oxidative stress are induced by HMG and MGA in heart, which may explain the cardiac dysfunction observed in HL deficiency, and also suggest that antioxidant supplementation could be considered as adjuvant therapy for affected patients. 相似文献
4.
Fabiany da Costa Gonçalves Mateus Grings Natália Schneider Nunes Fernanda Otesbelgue Pinto Tuane Nerissa Alves Garcez Fernanda Visioli Guilhian Leipnitz Ana Helena Paz 《Biotechnology letters》2017,39(4):613-622
Objective
To investigate the effects of oxidative stress injury in dextran sulfate sodium (DSS)-induced colitis in mice treated with mesenchymal stem cells (MSC).Results
Mice exposed to oral administration of 2% DSS over 7 days presented a high disease activity index and an intense colonic inflammation. Systemic infusion of MSC protected from severe colitis, reducing weight loss and diarrhea while lowering the infiltration of inflammatory cells. Moreover, toxic colitis injury increased oxidative stress. Administration of DSS decreased reduced glutathione (GSH) and superoxide dismutase (SOD) activity, and increased thiobarbituric acid-reactive substances levels in the colon. No alteration was found in catalase (CAT) and glutathione peroxidase (GPx) activity. Otherwise, MSC transplantation was able to prevent the decrease of GSH levels and SOD activity suggestive of an antioxidant property of MSC.Conclusion
The oxidative stress is a pathomechanism underlying the pathophysiology of colitis and MSC play an important role in preventing the impairment of antioxidants defenses in inflamed colon.5.
Chester Bittencourt Sacramento Vanessa Dionisio Cantagalli Mariana Grings Leonardo Pinto Carvalho José Carlos Costa Baptista‐Silva Abram Beutel Cassia Toledo Bergamaschi Ruy Ribeiro de Campos Junior Jane Zveiter de Moraes Christina Maeda Takiya Vívian Yochiko Samoto Radovan Borojevic Flavia Helena da Silva Nance Beyer Nardi Hans Fernando Dohmann Hamilton Silva Junior Valderez Bastos Valero Sang Won Han 《The journal of gene medicine》2009,11(4):345-353
6.
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
7.
Anelise M. Tonin Alexandre U. Amaral Estela N. B. Busanello Mateus Grings Roger F. Castilho Moacir Wajner 《Journal of bioenergetics and biomembranes》2013,45(1-2):47-57
Cardiomyopathy is a common clinical feature of some inherited disorders of mitochondrial fatty acid β-oxidation including mitochondrial trifunctional protein (MTP) and isolated long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiencies. Since individuals affected by these disorders present tissue accumulation of various fatty acids, including long-chain 3-hydroxy fatty acids, in the present study we investigated the effect of 3-hydroxydecanoic (3 HDCA), 3-hydroxydodecanoic (3 HDDA), 3-hydroxytetradecanoic (3 HTA) and 3-hydroxypalmitic (3 HPA) acids on mitochondrial oxidative metabolism, estimated by oximetry, NAD(P)H content, hydrogen peroxide production, membrane potential (ΔΨ) and swelling in rat heart mitochondrial preparations. We observed that 3 HTA and 3 HPA increased resting respiration and diminished the respiratory control and ADP/O ratios using glutamate/malate or succinate as substrates. Furthermore, 3 HDDA, 3 HTA and 3 HPA decreased ΔΨ, the matrix NAD(P)H pool and hydrogen peroxide production. These data indicate that these fatty acids behave as uncouplers of oxidative phosphorylation. We also verified that 3 HTA-induced uncoupling-effect was not mediated by the adenine nucleotide translocator and that this fatty acid induced the mitochondrial permeability transition pore opening in calcium-loaded organelles since cyclosporin A prevented the reduction of mitochondrial ΔΨ and swelling provoked by 3 HTA. The present data indicate that major 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies behave as strong uncouplers of oxidative phosphorylation potentially impairing heart energy homeostasis. 相似文献
8.
9.
?ngela Zanatta Alana Pimentel Moura Anelise Miotti Tonin Lisiane Aur��lio Knebel Mateus Grings Vannessa Ara��jo Lobato C��sar Augusto Jo?o Ribeiro Carlos Severo Dutra-Filho Guilhian Leipnitz Moacir Wajner 《Cellular and molecular neurobiology》2013,33(1):137-146
Isolated 3-methylcrotonyl-CoA carboxylase deficiency (3MCCD) is an autosomal recessive disorder of leucine metabolism biochemically characterized by accumulation of 3-methylcrotonylglycine (3MCG), 3-methylcrotonic acid (3MCA) and 3-hydroxyisovaleric acid. A considerable number of affected individuals present neurological symptoms with or without precedent crises of metabolic decompensation and brain abnormalities whose pathogenesis is poorly known. We investigated the in vitro effects of 3MCG and 3MCA on important parameters of oxidative stress in cerebral cortex of young rats. 3MCG and 3MCA significantly increased TBA-RS and carbonyl formation, indicating that these compounds provoke lipid and protein oxidation, respectively. In contrast, nitric oxide production was not affected by 3MCG and 3MCA. Furthermore, 3MCG- and 3MCA-induced elevation of TBA-RS values was fully prevented by melatonin, trolox and reduced glutathione, but not by the nitric oxide inhibitor N??-nitro-l-arginine methyl ester or the combination of catalase plus superoxide dismutase, indicating that reactive oxygen species were involved in the oxidative damage caused by these compounds. We also found that the activity of the antioxidant enzymes glutathione peroxidase, catalase, superoxide dismutase and glutathione reductase were not altered in vitro by 3MCG and 3MCA. It is therefore presumed that alterations of the cellular redox homeostasis caused by the major metabolites accumulating in 3MCCD may potentially be involved in the pathophysiology of the neurological dysfunction and structural brain alterations found in patients affected by this disorder. 相似文献
10.
Patrícia Fernanda Schuck Estela Natacha Brandt Busanello Alana Pimentel Moura Anelise Miotti Tonin Mateus Grings Luciana Ritter Carmen Regla Vargas Gustavo da Costa Ferreira Moacir Wajner 《Neurochemical research》2010,35(2):298-305
High concentrations of ethylmalonic acid are found in tissues and biological fluids of patients affected by ethylmalonic encephalopathy,
deficiency of short-chain acyl-CoA dehydrogenase activity and other illnesses characterized by developmental delay and neuromuscular
symptoms. The pathophysiological mechanisms responsible for the brain damage in these patients are virtually unknown. Therefore,
in the present work we investigated the in vitro effect of EMA on oxidative stress parameters in rat cerebral cortex. EMA
significantly increased chemiluminescence and thiobarbituric acid-reactive species levels (lipoperoxidation), as well as carbonyl
content and oxidation of sulfhydryl groups (protein oxidative damage) and DCFH. EMA also significantly decreased the levels
of reduced glutathione (non-enzymatic antioxidant defenses). In contrast, nitrate and nitrite levels were not affected by
this short organic acid. It is therefore presumed that oxidative stress may represent a pathomechanism involved in the pathophysiology
of the neurologic symptoms manifested by patients affected by disorders in which EMA accumulates. 相似文献