# From mice to men: lessons from mutant ataxic mice

## TL;DR

This paper reviews various ataxic mouse models to understand cerebellar degenerative disorders and their potential for studying treatment approaches.

## Contribution

The paper provides a comprehensive review of multiple mouse models and their relevance to human cerebellar diseases.

## Key findings

- Mouse models like Lurcher and Staggerer show distinct cerebellar pathologies and functional changes.
- Several models, including SCA1 and Friedreich ataxia, help study the pathogenesis of human ataxic disorders.
- The paper highlights both the benefits and limitations of using mouse models for therapeutic research.

## 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.