Spinocerebellar ataxia type 1 (SCA1) is an inherited neurodegenerative disease caused by expansion of a glutamine-encoding repeat in ataxin 1. The expanded polyglutamine tract can affect the function of the ataxin1 protein in different ways, depending on the protein partners ataxin1 is associated with. This paper shows that polyglutamine expansion in one and the same protein can cause gain of function and loss of function toxicity at the same time.
- Janghoo Lim
- Juan Crespo-Barreto
- Huda Y. Zoghbi