MOUSE MODELS OF HUMAN CAG REPEAT DISORDERS

Expansions of CAG trinucleotide repeats encoding glutamine have been f ound to be the causative mutations of seven human neurodegenerative di seases, Similarities in the clinical, genetic, and molecular features of these disorders suggest they share a common mechanism of pathogenes is, Recent progress in the generation and characterization of transgen ic mice expressing the genes containing expanded repeats associated wi th spinal and bulbar muscular atrophy (SBMA), spinocerebellar ataxia t ype 1 (SCA1), Machado-Joseph disease (MJD/SCA3), and Huntington's dise ase (HD) is beginning to provide insight into the underlying mechanism s of these neurodegenerative disorders.