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GERSTMANN–Sträussler syndrome is a rare familial neuro-degenerative condition that is vertically transmitted, in an apparently autosomal dominant way¹. It can also be horizontally transmitted to non-human primates and rodents through intracerebral inoculation of brain homogenates from patients with the disease^2–8. The exact incidence of the syndrome is unknown but is estimated to be between one and ten per hundred million. Patients initially suffer from ataxia or dementia and deteriorate until they die, in one to ten years. Protease-resistant prion protein (PrP) and PrP-immunoreactive amyloid plaques with characteristic morphology accumulate in the brains of these patients^9–11. Current diagnostic criteria for Gerstmann–Sträussler syndrome incorporate clinical and neuropathological features, as animal transmission studies can be unreliable^8,12. PrP is implicated in the pathogenesis and transmission of the …