Article
Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.
Brain : a journal of neurology - 1 Jun 2013
Chort Alice, Alves Sandro, Marinello Martina, Dufresnois Béatrice, Dornbierer Jean-Gabriel, Tesson Christelle, Latouche Morwena, Baker Darren P, Barkats Martine, El Hachimi Khalid H, Ruberg Merle, Janer Alexandre, Stevanin Giovanni, Brice Alexis, Sittler Annie
Abstract excerpt
We showed previously, in a cell model of spinocerebellar ataxia 7, that interferon beta induces the expression of PML protein and the formation of PML protein nuclear bodies that degrade mutant ataxin 7, suggesting that the cytokine, used to treat multiple sclerosis, might have therapeutic value in spinocerebellar ataxia 7. We now show that interferon beta also induces PML-dependent clearance of ataxin 7 in a...
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