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Unstable expansion of triplet repeats as a new disease mechanism for neurodegenerative diseases

1996; Springer Science+Business Media; Volume: 41; Issue: 3 Linguagem: Inglês

10.1007/bf01913170

0021-5074

S Tsuji,

Ubiquitin and proteasome pathways

As discussed in the present review, many challenging issues regarding the molecular mechanisms of neurodegeneration are associated with investigation of triplet repeat diseases. Since the molecular structures of the causative genes for triplet repeat diseases have been fully elucidated, triplet repeat diseases no doubt offer the best model systems to investigate the pathophysiology of neurodegenerative diseases, and it is strongly expected that creation of animal models of human diseases and development of cell culture systems for investigation of neuronal toxicity caused by triplet repeat expansions will be a crucial step for development of therapeutic measures for triplet repeat diseases.