Autor(es): Santana, Magda M. ; Paixão, Susana ; Cunha-Santos, Janete ; Silva, Teresa Pereira ; Trevino-Garcia, Allyson ; Gaspar, Laetitia S. ; Nóbrega, Clévio ; Nobre, Rui Jorge ; Cavadas, Cláudia ; Greif, Hagar ; Pereira de Almeida, Luís
Data: 2020
Identificador Persistente: http://hdl.handle.net/10400.1/13814
Origem: Sapientia - Universidade do Algarve
Assunto(s): Machado-Joseph disease; Autophagy; Spinocerebellar ataxia type 3; Polyglutamine disorder; Trehalose
Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration. There is no treatment available to block or delay disease progression. In this work we investigated whether trehalose, a natural occurring disaccharide widely used in food and cosmetic industry, would rescue biochemical, behavioral and neuropathological features of an in vitro and of a severe MJD transgenic mouse model.
