Article
Neuropathology of Gerstmann-Sträussler-Scheinker disease.
Microscopy research and technique - 1 Jul 2000
Bugiani O, Giaccone G, Piccardo P, Morbin M, Tagliavini F, Ghetti B
Abstract excerpt
Gerstmann-Sträussler-Scheinker disease is a familial neurodegeneration characterized clinically by adult-onset ataxia, postural abnormalities, and cognitive decline, and pathologically by amyloid deposits mostly localized in the cerebral and cerebellar cortices and the basal ganglia. The disease is due to mutations in the prion protein gene. Processing of the mutant proteins originates the amyloidogenic fragments...
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