Prinses Beatrix Fonds grant to study fate of toxic (CUG)n RNA in myotonic dystrophy

Wansink Rick

Myotonic dystrophy type 1 is the most prevalent form of muscular dystrophy in adults. The Cell Biology Department, as part of a consortium, discovered almost twenty years ago that the disease is caused by an expanding (CTG)n repeat in the 3' non-coding region of the DMPK gene.

From that time on, the molecular pathogenesis underlying the complex, multisystemic features has been a continued topic in the Department of Cell Biology. Rick Wansink and Be Wieringa now received a 250 kEUR grant from the Prinses Beatrix Fonds to help unravel the fate of expanded DMPK RNA - characterized by a stable, toxic (CUG)n hairpin structure - in nucleus and cytosol.

Expanded DMPK RNA will be followed from "birth to decay" using molecular cell biological and state-of-the-art microscopical techniques. Spatiotemporal and quantitative aspects of (CUG)n RNA metabolism will be examined in absence and presence of potential therapeutics.

Photo: Rick Wansink


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