First Mouse Model for ALS-dementia Developed

In 2011, ALS-causing mutations in ubiquilin2 (UBQLN2) were first identified, providing a direct link between the proteasomal degradation pathway and ALS (see Aug 2011 news story). In a report in the September 22 Proceedings of the National Academy of Sciences, the same team, led by Teepu Siddique at the Northwestern University Feinberg School of Medicine in Chicago, IL, described a new mouse model carrying these mutations. The model recapitulates behavioral, neurophysiological and pathological changes that are observed in human ALS-dementia. The mouse model will facilitate examination of brain activity in living mice, as well as serve as a new tool to test candidate therapies in ALS and ALS-dementia. Click here to read more.

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