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C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

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Science  05 Sep 2014:
Vol. 345, Issue 6201, pp. 1192-1194
DOI: 10.1126/science.1256800

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Dipeptide repeat peptides on the attack

Certain neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), are associated with expanded dipeptides translated from RNA transcripts of disease-associated genes (see the Perspective by West and Gitler). Kwon et al. show that the peptides encoded by the expanded repeats in the C9orf72 gene interfere with the way cells make RNA and kill cells. These effects may account for how this genetic form of ALS causes disease. Working in Drosophila, Mizielinska et al. aimed to distinguish between the effects of repeat-containing RNAs and the dipeptide repeat peptides that they encode. The findings provide evidence that dipeptide repeat proteins can cause toxicity directly.

Science, this issue p. 1139 and p. 1192; see also p. 1118