NEURODEGENERATIVE DISEASE:C9orf72repeats compromise nucleocytoplasmic transport

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Abstract

The molecular mechanisms of neurodegeneration due to a repeat expansion inC9orf72, the most common cause of frontotemporal dementia and amyotrophic lateral sclerosis, are unknown. Three reports now link compromised nucleocytoplasmic transport to disease pathogenesis. Whether RNA structures or dipeptide repeat proteins are most toxic in humans remains open to question.

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