Výsledky vyhledávání - "Leonardo de Moura Alvorcem"

Evidence that thiol group modification and reactive oxygen species are involved in hydrogen sulfide-induced mitochondrial permeability transition pore opening in rat cerebellum

Autor: Guilhian Leipnitz, Belisa Parmeggiani, Cristiane Cecatto, Renata Britto, Alexandre Umpierrez Amaral, Larissa Daniele Bobermin, Moacir Wajner, Nícolas Manzke Glänzel, Nevton Teixeira da Rosa-Junior, Leonardo de Moura Alvorcem

Publikováno v: Mitochondrion. 47:141-150

We report here the effects of hydrogen sulfide (sulfide), that accumulates in ETHE1 deficiency, in rat cerebellum. Sulfide impaired electron transfer and oxidative phosphorylation. Sulfide also induced mitochondrial swelling, and decreased ΔΨm and

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::debc77d22adba524a2991836f01fa287
https://doi.org/10.1016/j.mito.2018.11.001

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Antioxidant system disturbances and mitochondrial dysfunction induced by 3-methyglutaric acid in rat heart are prevented by bezafibrate

Autor: Nevton Teixeira da Rosa-Junior, Belisa Parmeggiani, Nícolas Manzke Glänzel, Leonardo de Moura Alvorcem, Morgana Brondani, Renata Britto, Mateus Grings, Vanessa Duarte Ortiz, Patrick Turck, Alex Sander da Rosa Araujo, Moacir Wajner, Guilhian Leipnitz

Publikováno v: European Journal of Pharmacology. 924:174950

Barth syndrome (BTHS) and dilated cardiomyopathy with ataxia syndrome (DCMA) are biochemically characterized by high levels of 3-methylglutaric acid (MGA) in the urine and plasma of affected patients. Although cardiolipin abnormalities have been obse

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::20ad899cb732e2450de0d289a214e91a
https://doi.org/10.1016/j.ejphar.2022.174950

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In vivo evidence that bezafibrate prevents oxidative stress and mitochondrial dysfunction caused by 3-methylglutaric acid in rat liver

Autor: Belisa Parmeggiani, Guilhian Leipnitz, Marina Rocha Frusciante, Nevton Teixeira da Rosa-Junior, Carlos Severo Dutra Filho, Leonardo de Moura Alvorcem, Nícolas Manzke Glänzel, Moacir Wajner

Publikováno v: Biochimie.

High urinary excretion and tissue accumulation of 3-methylglutaric acid (MGA) are observed in patients affected by 3-hydroxy-3-methylglutaric (HMGA) and 3-methylglutaconic (MGTA) acidurias. The pathomechanisms underlying the hepatic dysfunction commo

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b5553a353ae86743133acf4c40cde7cf
https://pubmed.ncbi.nlm.nih.gov/32169667

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3-Hydroxy-3-Methylglutaric Acid Impairs Redox and Energy Homeostasis, Mitochondrial Dynamics, and Endoplasmic Reticulum-Mitochondria Crosstalk in Rat Brain

Autor: Guilhian Leipnitz, Belisa Parmeggiani, Nevton Teixeira da Rosa-Junior, Mateus Struecker da Rosa, Rafael Teixeira Ribeiro, Leonardo de Moura Alvorcem, Nícolas Manzke Glänzel, Mateus Grings, Moacir Wajner

Publikováno v: Neurotoxicity research. 37(2)

3-Hydroxy-3-methylglutaryl-CoA lyase (HL) deficiency is a neurometabolic disorder characterized by predominant accumulation of 3-hydroxy-3-methylglutaric acid (HMG) in tissues and biological fluids. Patients often present in the first year of life wi

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::990d6ca047b2d7cdbd1ed59a9d74f771
https://pubmed.ncbi.nlm.nih.gov/31721046

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Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats

Autor: Guilhian Leipnitz, Moacir Wajner, Belisa Parmeggiani, Mateus Struecker da Rosa, Nícolas Manzke Glänzel, Nevton Teixeira da Rosa-Junior, Leonardo de Moura Alvorcem

Publikováno v: Neurotoxicity research. 35(4)

3-Methylglutaric acid (MGA) is an organic acid that accumulates in 3-methylglutaconic (MGTA) and 3-hydroxy-3-methylglutaric (HMGA) acidurias. Patients affected by these disorders present with neurological dysfunction that usually appears in the first

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::76e812d80b4b198acbfee029eff56cb1
https://pubmed.ncbi.nlm.nih.gov/30850947

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Evidence that Thiosulfate Inhibits Creatine Kinase Activity in Rat Striatum via Thiol Group Oxidation

Autor: Moacir Wajner, Mateus Grings, Alana Pimentel Moura, Angela T. S. Wyse, Leonardo de Moura Alvorcem, Belisa Parmeggiani, Guilhian Leipnitz

Publikováno v: Neurotoxicity research. 34(3)

Sulfite oxidase, molybdenum cofactor, and ETHE1 deficiencies are autosomal recessive disorders that affect the metabolism of sulfur-containing amino acids. Patients with these disorders present severe neurological dysfunction and basal ganglia abnorm

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f39213b5d440743af3fe65ec13ad7c1d
https://pubmed.ncbi.nlm.nih.gov/30056533

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Intracerebral Glycine Administration Impairs Energy and Redox Homeostasis and Induces Glial Reactivity in Cerebral Cortex of Newborn Rats

Autor: Guilhian Leipnitz, Alana Pimentel Moura, Mateus Grings, Leonardo de Moura Alvorcem, Belisa Parmeggiani, Moacir Wajner, Bianca Seminotti, Marcela Moreira Motta, Anna Paula Bumbel, Rafael Mello Boldrini

Publikováno v: Molecular Neurobiology. 53:5864-5875

Accumulation of glycine (GLY) is the biochemical hallmark of glycine encephalopathy (GE), an aminoacidopathy characterized by severe neurological dysfunction that may lead to early death. In the present study, we evaluated the effect of a single intr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1343b39b3d142973513038126c24d7ee
https://doi.org/10.1007/s12035-015-9493-7

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Disruption of Energy Transfer and Redox Status by Sulfite in Hippocampus, Striatum, and Cerebellum of Developing Rats

Autor: Guilhian Leipnitz, Belisa Parmeggiani, Moacir Wajner, Felipe Schmitz, Angela T. S. Wyse, Mateus Grings, Nícolas Manzke Glänzel, Leonardo de Moura Alvorcem, Mateus Struecker da Rosa

Publikováno v: Neurotoxicity research. 32(2)

Patients with sulfite oxidase (SO) deficiency present severe brain abnormalities, whose pathophysiology is not yet elucidated. We evaluated the effects of sulfite and thiosulfate, metabolites accumulated in SO deficiency, on creatine kinase (CK) acti

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5eddca22884d692721a3c0f225854238
https://pubmed.ncbi.nlm.nih.gov/28417315

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2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain

Autor: Guilhian Leipnitz, Moacir Wajner, Anelise Miotti Tonin, Lisiane Aurélio Knebel, Leonardo de Moura Alvorcem, Ângela Zanatta, Mateus Grings

Publikováno v: Brain Research. 1478:74-82

Short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency is an autosomal recessive disorder of isoleucine metabolism biochemically characterized by accumulation of 2-methylbutyrylglycine (2MBG) and 2-methylbutyric acid (2MB). Affected patients

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4280dc86f2b6c30049e3779d8b443de3
https://doi.org/10.1016/j.brainres.2012.08.039

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