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	Provedor de dados BJMBR (1) Genet. Mol. Biol. (1) Autor Bolzan,Gabriela (1) Dogini,D.B. (1) Furtado,Gabriel Vasata (1) Gilioli,R. (1) Jardim,Laura Bannach (1) Lopes-Cendes,I. (1) Lopes-Ramos,C.M. (1) Oliveira,Camila Maria de (1) Pereira,T.C. (1) Saraiva-Pereira,Maria Luiza (1) Saute,Jonas Alex Morales (1) Mais... Palavra-chave Machado-Joseph disease (2) Spinocerebellar ataxia type 3 (2) Biomarkers (1) Coenzyme Q10 (1) Drug treatment (1) Lithium carbonate (1) Neurophysiology (1) Mais... Tipo do documento Info:eu-repo/semantics/article (2) Ano 2019 (1) 2016 (1) País Brazil (2) Idioma Inglês (2)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 2 Primeira ... 1 ... Última Lithium carbonate and coenzyme Q10 reduce cell death in a cell model of Machado-Joseph disease BJMBR Lopes-Ramos,C.M.; Pereira,T.C.; Dogini,D.B.; Gilioli,R.; Lopes-Cendes,I.. Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disorder caused by expansion of the polyglutamine domain of the ataxin-3 (ATX3) protein. MJD/SCA3 is the most frequent autosomal dominant ataxia in many countries. The mechanism underlying MJD/SCA3 is thought to be mainly related to protein misfolding and aggregation leading to neuronal dysfunction followed by cell death. Currently, there are no effective treatments for patients with MJD/SCA3. Here, we report on the potential use of lithium carbonate and coenzyme Q10 to reduce cell death caused by the expanded ATX3 in cell culture. Cell viability and apoptosis were evaluated by MTT assay and by flow cytometry after staining with annexin... Tipo: Info:eu-repo/semantics/article Palavras-chave: Machado-Joseph disease; Spinocerebellar ataxia type 3; Lithium carbonate; Coenzyme Q10; Drug treatment. Ano: 2016 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2016001200601 State biomarkers for Machado Joseph disease: Validation, feasibility and responsiveness to change Genet. Mol. Biol. Furtado,Gabriel Vasata; Oliveira,Camila Maria de; Bolzan,Gabriela; Saute,Jonas Alex Morales; Saraiva-Pereira,Maria Luiza; Jardim,Laura Bannach. Abstract Machado-Joseph disease (SCA3/MJD) is the most common spinocerebellar ataxia worldwide, and particularly so in Southern Brazil. Due to an expanded polyglutamine at ataxin-3, SCA3/MJD presents a relentless course with no current disease modifying treatment. Clinical scales used to measure SCA3/MJD progression present moderate effect sizes, a major drawback for their use as main outcomes in clinical trials, given the rarity and slow progression of the disease. This limitation might be overcome by finding good surrogate markers. We present here a review of studies on peripheral and neurophysiological markers in SCA3/MJD that can be candidates for state biomarkers. Data on markers already studied were summarized, giving emphasis on validation against... Tipo: Info:eu-repo/semantics/article Palavras-chave: Biomarkers; Neurophysiology; Machado-Joseph disease; Spinocerebellar ataxia type 3. Ano: 2019 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572019000200238 Registros recuperados: 2 Primeira ... 1 ... Última

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