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Journal / Neuroscience / A Simple Composite Phenotype Scoring System for…
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JoVE Journal Neuroscience
A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
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A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia

Article DOI: 10.3791/1787-v • 07:33 min • May 21st, 2010
May 21st, 2010 •
1, 2, 1, 2, *3, *2
1Department of Biochemistry, University of Washington, 2Department of Neurology, University of Washington, 3Division of Genetics, Departments of Pediatrics and Cellular and Molecular Medicine, and the Institute for Genomic Medicine, University of California, San Diego - Rady Children’s Hospital
* These authors contributed equally

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We describe a protocol for the rapid and sensitive quantification of disease severity in mouse models of cerebellar ataxia. Measures include hind limb clasping, ledge test, gait and kyphosis. This protocol effectively discriminates between affected and non-affected individuals, and detects the progression of affected individuals over time.

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Composite Phenotype Scoring System Mouse Models Cerebellar Ataxia Phenotype Assessments Disease Severity Hind Limb Clasping Ledge Test Gait Kyphosis Neurodegenerative Disease Phenotypes Temporal Progression Transgenic Mouse Model Spinocerebellar Ataxia Type 7 (SCA7) Albert R. La Spada Gwenn A. Garden
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