Model systems are important tools for the investigation of pathogenic processes. Especially for diseases with a late onset of symptoms and slow progression, like most spinocerebellar ataxias (SCA), it is time-consuming or even impossible to analyze all aspects of the pathogenesis in humans. Due to the reduced lifespan of model organisms, it is possible to study disease progression in full within a reasonable timeframe and due to the shorter generation time of most model organisms more individuals can be generated and analyzed, thereby strengthening the reliability of data via an increased number of replicates. Detailed studies of the histopathology can only be performed as endpoint analyses in.
