Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model

Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model

Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited cerebellar ataxia caused by the expansion of a polyglutamine (polyQ) repeat in the gene encoding ATXN3. The polyQ expansion induces protein inclusion formation in the neurons of patients and results in neuronal degeneration in the cerebe...

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Bibliographic Details
Main Authors: Karen Jansen-West, Tiffany W. Todd, Lillian M. Daughrity, Mei Yue, Jimei Tong, Yari Carlomagno, Giulia Del Rosso, Aishe Kurti, Caroline Y. Jones, Judith A. Dunmore, Monica Castanedes-Casey, Dennis W. Dickson, Zbigniew K. Wszolek, John D. Fryer, Leonard Petrucelli, Mercedes Prudencio
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-03-01
Series:Frontiers in Cell and Developmental Biology
Subjects:
spinocerebellar ataxia 3
SCA3
Machado-Joseph disease
ATXN3
polyglutamine
mouse model
Online Access:https://www.frontiersin.org/articles/10.3389/fcell.2022.863089/full

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https://www.frontiersin.org/articles/10.3389/fcell.2022.863089/full

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