Abstract
Spinocerebellar ataxias are neurodegenerative cerebellar disorders with autosomal dominant heredity. Spinocerebellar ataxia type-2 (SCA2) is caused by expansion of CAG repeat in the ataxin-2 gene.
Several mouse transgenic models of the SCA2 have been generated by insertion of gene with enlarged repeat. They serve for investigation of SCA2 patogenesis and its therapy.
Individual lines of SCA2 mice differ in number of CAG repetitions in the transgene, strain of origin and probably also in localisation of the transgene insertion. Basic manifestation is given by cerebellar degeneration and involve first of all motor deficit.
Its progress depends on above mentioned characteristics of the particular SCA2 mouse line.