Reduction of mutant ATXN1 rescues premature death in a conditional SCA1 mouse model

Spinocerebellar ataxia type 1 (SCA1) is an adult-onset neurodegenerative disorder. As disease progresses, motor neurons are affected, and their dysfunction contributes toward the inability to maintain proper respiratory function, a major driving force for premature death in SCA1. To investigate the...

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Bibliographic Details
Main Authors: James P. Orengo, Larissa Nitschke, Meike E. van der Heijden, Nicholas A. Ciaburri, Harry T. Orr, Huda Y. Zoghbi
Format: Article
Language:English
Published: American Society for Clinical investigation 2022-04-01
Series:JCI Insight
Subjects:
Online Access:https://doi.org/10.1172/jci.insight.154442