Ataxic mutant mice can be used to represent models of cerebellar degenerative disorders. They serve for investigation of cerebellar function, pathogenesis of degenerative processes as well as of therapeutic approaches. Lurcher, Hot-foot, Purkinje cell degeneration, Nervous, Staggerer, Weaver, Reeler, and Scrambler mouse models and mouse models of SCA1, SCA2, SCA3, SCA6, SCA7, SCA23, DRPLA, Niemann-Pick disease and Friedreich ataxia are reviewed with special regard to cerebellar pathology, pathogenesis, functional changes and possible therapeutic influences, if any. Finally, benefits and limitations of mouse models are discussed.

Department of Pathophysiology Faculty of Medicine in Pilsen Charles University Prague Lidicka 1 301 66 Plzen Czech Republic ; Biomedical Centre Faculty of Medicine in Pilsen Charles University Prague Plzen Czech Republic

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Lurcher Mouse as a Model of Cerebellar Syndromes

Reduction of Microvessel Number and Length in the Cerebellum of Purkinje Cell Degeneration Mice

Consensus Paper: Strengths and Weaknesses of Animal Models of Spinocerebellar Ataxias and Their Clinical Implications

Cerebellum-from J. E. Purkyně up to Contemporary Research

Smaller Absolute Quantities but Greater Relative Densities of Microvessels Are Associated with Cerebellar Degeneration in Lurcher Mice

Experimental neurotransplantation treatment for hereditary cerebellar ataxias

Transplantation of Embryonic Cerebellar Grafts Improves Gait Parameters in Ataxic Lurcher Mice

Mutation-related differences in exploratory, spatial, and depressive-like behavior in pcd and Lurcher cerebellar mutant mice