Article
Similarities between spinocerebellar ataxia type 7 (SCA7) cell models and human brain: proteins recruited in inclusions and activation of caspase-3.
Human molecular genetics - 15 Oct 2001
Zander C, Takahashi J, El Hachimi K H, Fujigasaki H, Albanese V, Lebre A S, Stevanin G, Duyckaerts C, Brice A
Abstract excerpt
Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant polyglutamine disorder presenting with progressive cerebellar ataxia and blindness. The molecular mechanisms underlying the selective neuronal death typical of SCA7 are unknown. We have established SCA7 cell culture models in HEK293 and SH-SY5Y cells, in order to analyse the effects of overexpression of the mutant ataxin-7 protein. The cells readily...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.