Zobrazeno 1 - 10
of 13
pro vyhledávání: '"Nevton Teixeira da Rosa-Junior"'
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
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
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
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
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
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
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
Autor:
da Rosa, Mateus Struecker1 (AUTHOR), da Rosa-Junior, Nevton Teixeira1 (AUTHOR), Parmeggiani, Belisa1 (AUTHOR), Glänzel, Nícolas Manzke1 (AUTHOR), de Moura Alvorcem, Leonardo1 (AUTHOR), Ribeiro, Rafael Teixeira1 (AUTHOR), Grings, Mateus1 (AUTHOR), Wajner, Moacir2,3 (AUTHOR), Leipnitz, Guilhian2,4 (AUTHOR) guilhian@ufrgs.br
Publikováno v:
Neurotoxicity Research. Feb2020, Vol. 37 Issue 2, p314-325. 12p.
Autor:
da Rosa-Junior, Nevton Teixeira1 (AUTHOR), Parmeggiani, Belisa1 (AUTHOR), da Rosa, Mateus Struecker1 (AUTHOR), Glänzel, Nícolas Manzke1 (AUTHOR), de Moura Alvorcem, Leonardo1 (AUTHOR), Wajner, Moacir1,2,3 (AUTHOR), Leipnitz, Guilhian1,2 (AUTHOR) guilhian@ufrgs.br
Publikováno v:
Neurotoxicity Research. May2019, Vol. 35 Issue 4, p809-822. 14p.
Autor:
Glänzel, Nícolas Manzke, Grings, Mateus, da Rosa‐Junior, Nevton Teixeira, Carvalho, Leila Maria Cereta, Mohsen, Al‐Walid, Wipf, Peter, Wajner, Moacir, Vockley, Jerry, Leipnitz, Guilhian
Publikováno v:
Journal of Inherited Metabolic Disease; Mar2021, Vol. 44 Issue 2, p481-491, 11p