Lecture: Visiting Professor Alanna Watt
Lecture by Visiting Professor Alanna Watt, McGill University, Montreal, Canada
Understanding and reversing pathophysiological changes in a mouse model of ataxia
Spinocerebellar ataxia type 6 (SCA6) is a midlife-onset degenerative disease affecting motor coordination and balance. Using mouse transgenics, behavioural, electrophysiological, and imaging techniques, the Watt lab studies the cellular changes that contribute to disease onset and progression in SCA6 and other ataxias.
Cerebellar Purkinje cells undergo cell death at late stages of disease progression in SCA6 mice. However, changes in Purkinje cell firing properties are observed earlier, at disease onset. We have recently identified two distinct interventions that both partially rescue motor performance in SCA6 mice: one pharmacological (the FDA-approved drug 4-aminopyridine) and one behavioural. Both interventions rescue distinct firing deficits in SCA6 cerebellar Purkinje cells, suggesting that their combined effect may be additive. These results provide novel therapeutic approaches for understanding and treating ataxia in patients.
Host:Contact person: Eva Hedlund