Abnormal repeats in the polyglutamine protein Ataxin-2 cause spinocerebellar ataxia type 2 and increase ALS disease susceptibility (see Aug 2010 news story and Aug 2013 news story). How exactly these mutations cause disease is not well understood. Now, researchers from University of Toronto in Ontario, Canada led by Karim Mekhail have taken a major step in this direction by studying Pbp1, the yeast ortholog of Ataxin-2. In the study published July 28 in Developmental Cell, the investigators demonstrate that Pbp1 inhibits the formation of non-coding DNA-RNA hybrids and prevents aberrant recombination. Caloric restriction in Pbp1-null yeast suppresses the accumulation of RNA-DNA hybrids. The scientists plan to replicate these findings in tissue from ALS patients and test whether caloric restriction is beneficial for ALS patients. Click here to read more.
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