From mice to men: lessons from mutant ataxic mice.
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 in Prague, Lidicka 1, 301 66 Plzen, Czech Republic ; Biomedical Centre, Faculty of Medicine in Pilsen, Charles University in Prague, Plzen, Czech Republic.
Jan
J
Cendelin
Ataxia; Cerebellum; Neurodegeneration