Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin.

Duncan, Emma J; Larivière, Roxanne; Bradshaw, Teisha Y; Longo, Fabiana; Sgarioto, Nicolas; Hayes, Matthew J; Romano, Lisa E L; Nethisinghe, Suran; Giunti, Paola; Bruntraeger, Michaela B; Durham, Heather D; Brais, Bernard; Maltecca, Francesca; Gentil, Benoit J; Chapple, J Paul

Duncan, Emma J; Larivière, Roxanne; Bradshaw, Teisha Y; Longo, Fabiana; Sgarioto, Nicolas; Hayes, Matthew J; Romano, Lisa E L; Nethisinghe, Suran; Giunti, Paola; Bruntraeger, Michaela B; Durham, Heather D; Brais, Bernard; Maltecca, Francesca; Gentil, Benoit J; Chapple, J Paul.

Afiliação

Duncan EJ; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Larivière R; Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, QC H3A 2B4, Canada.
Bradshaw TY; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Longo F; Università Vita-Salute San Raffaele and Division of Genetics and Cell Biology, San Raffaele Scientific Institute, Milan, Italy.
Sgarioto N; Università degli Studi dell'Insubria, Varese, Italy.
Hayes MJ; Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, QC H3A 2B4, Canada.
Romano LEL; UCL Institute of Ophthalmology, London EC1V 9E, UK.
Nethisinghe S; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Giunti P; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Bruntraeger MB; Department of Molecular Neuroscience, UCL Institute of Neurology, London WC1N 3BG, UK.
Durham HD; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Brais B; Laboratory of Neurogenetics of Motion, Montreal Neurological Institute, McGill University, Montreal, QC H3A 2B4, Canada.
Maltecca F; Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, QC H3A 2B4, Canada.
Gentil BJ; Università Vita-Salute San Raffaele and Division of Genetics and Cell Biology, San Raffaele Scientific Institute, Milan, Italy.
Chapple JP; Laboratory of Neurogenetics of Motion, Montreal Neurological Institute, McGill University, Montreal, QC H3A 2B4, Canada.

Hum Mol Genet ; 26(16): 3130-3143, 2017 08 15.

Article em En | MEDLINE | ID: mdl-28535259

Assuntos

Proteínas de Choque Térmico/metabolismo; Filamentos Intermediários/metabolismo; Animais; Ataxia/genética; Técnicas de Cultura de Células; Citoesqueleto/metabolismo; Fibroblastos/metabolismo; Proteínas de Choque Térmico HSP70/metabolismo; Proteínas de Choque Térmico/genética; Humanos; Proteína 2 de Membrana Associada ao Lisossomo/metabolismo; Camundongos; Mitocôndrias/metabolismo; Chaperonas Moleculares/metabolismo; Espasticidade Muscular/genética; Espasticidade Muscular/metabolismo; Proteostase/genética; Proteostase/fisiologia; Proteínas de Ligação a RNA/metabolismo; Ataxias Espinocerebelares/congênito; Ataxias Espinocerebelares/genética; Ataxias Espinocerebelares/metabolismo; Vimentina/metabolismo

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Filamentos Intermediários / Proteínas de Choque Térmico Limite: Animals / Humans Idioma: En Revista: Hum Mol Genet Ano de publicação: 2017 Tipo de documento: Article

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google