Insights into dentatorubral-pallidoluysian atrophy from a new Drosophila model of disease
Dentatorubral-pallidoluysian atrophy (DRPLA) is a polyglutamine neurodegenerative disorder caused by CAG expansions in ATN1. To investigate its pathogenesis, we developed and characterized  Drosophila models expressing human ATN1 with normal (Q7) or pathogenic (Q88) repeats. Overall design: Drosophila mdels expressing human ATN1 with normal (Q7) or pathogenic (Q88) repeats.