ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
oleh: Aarti Sharma, Alexander K. Lyashchenko, Lei Lu, Sara Ebrahimi Nasrabady, Margot Elmaleh, Monica Mendelsohn, Adriana Nemes, Juan Carlos Tapia, George Z. Mentis, Neil A. Shneider
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2016-02-01 |
Deskripsi
The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a loss or excess of FUS activity.