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FUS-Immunoreactive Intranuclear Inclusions in Neurodegenerative Disease
Brain Pathology, 10/16/09

Woulfe J et al. – The objective of the present study was to determine the range of neurodegenerative disorders characterized by FUS–positive NIIs. Immunostaining for FUS revealed intense reactivity of NIIs in FTLD–IF and FTLD–UPS as well as in Huntington's disease, spinocerebellar ataxias 1 and 3, and neuronal intranuclear inclusion body disease. In contrast, there was no FUS staining of NIIs in inherited forms of FTLD–TDP caused by GRN and VCP mutations, fragile–X–associated tremor ataxia syndrome, or oculopharyngeal muscular dystrophy. In a cell culture model of Huntington's disease, NIIs were intensely FUS–positive. NII–bearing cells displayed loss of the normal diffuse nuclear pattern of FUS staining. This suggests that sequestration of nuclear FUS by NIIs may interfere with its normal nuclear localization.

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