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Updated: Jan 24, 2026

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
Published on: May 21, 2010
Tag-Team Genetics of Spinocerebellar Ataxia 6
Eve-Ellen Govek1, Mary E Hatten1
1Laboratory of Developmental Neurobiology, The Rockefeller University, New York, NY 10065, USA.
Abstract:
In this issue of Neuron, Du et al. (2019) demonstrate that the bicistronic CACNA1A gene encodes a transcription factor α1ACT, mutations in which are associated with SCA6, that controls expression of genes important for cerebellar Purkinje cell development and excitability. Reduction of α1ACT in the adult is well tolerated, suggesting a potential new therapy for SCA6.
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