Výsledky vyhledávání - "Nevton Teixeira da Rosa-Junior"

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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The mitochondrial-targeted reactive species scavenger JP4-039 prevents sulfite-induced alterations in antioxidant defenses, energy transfer, and cell death signaling in striatum of rats

Autor: Nícolas Manzke Glänzel, Peter Wipf, Jerry Vockley, Moacir Wajner, Guilhian Leipnitz, Al-Walid Mohsen, Nevton Teixeira da Rosa-Junior, Mateus Grings, Leila Maria Cereta de Carvalho

Publikováno v: J Inherit Metab Dis

Sulfite oxidase (SO) deficiency is a disorder caused either by isolated deficiency of SO or by defects in the synthesis of its molybdenum cofactor. It is characterized biochemically by tissue sulfite accumulation. Patients present with seizures, prog

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fc0a6ae2bb509f15220f6894a123d209
https://pubmed.ncbi.nlm.nih.gov/32882059

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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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Bezafibrate Prevents Glycine-Induced Increase of Antioxidant Enzyme Activities in Rat Striatum

Autor: Renata Britto, Belisa Parmeggiani, Guilhian Leipnitz, Moacir Wajner, Mateus Grings, Nevton Teixeira da Rosa-Junior

Publikováno v: Molecular neurobiology. 56(1)

Non-ketotic hyperglycinemia (NKH) is a severe neurological disorder caused by defects in glycine (GLY) catabolism and characterized by a high cerebrospinal fluid/plasma GLY ratio. Treatment is often ineffective and limited to the control of symptoms

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::868874cc93f16313772db39db3f1edc3
https://pubmed.ncbi.nlm.nih.gov/29675575

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