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A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
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Neuroscienze
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JoVE Journal Neuroscienze
A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia

34,704 Views

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07:33 min

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May 21, 2010

DOI:

10.3791/1787-v

DOI:
10.3791/1787-v

•

07:33 min
May 21, 2010

•

38 Views
, , , , ,
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

Summary

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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 SystemMouse ModelsCerebellar AtaxiaPhenotype AssessmentsDisease SeverityHind Limb ClaspingLedge TestGaitKyphosisNeurodegenerative Disease PhenotypesTemporal ProgressionTransgenic Mouse ModelSpinocerebellar Ataxia Type 7 (SCA7)Albert R. La SpadaGwenn A. Garden

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Guyenet, S. J., Furrer, S. A., Damian, V. M., Baughan, T. D., La Spada, A. R., Garden, G. A. A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia. J. Vis. Exp. (39), e1787, doi:10.3791/1787 (2010).

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