[HTML][HTML] From mice to men: lessons from mutant ataxic mice

J Cendelin - Cerebellum & ataxias, 2014 - Springer
J Cendelin
Cerebellum & ataxias, 2014Springer
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 …
Abstract
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.
Springer