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Gerstmann-Sträussler-Scheinker disease. I. Human diseases.

Folia neuropathologica - 1 Jan 2004

Liberski Paweł P, Budka Herbert

Abstract excerpt

Gerstmann-Sträussler-Scheinker disease (GSS) is a slowly progressive hereditary autosomal dominant disease (OMIM: 137440) and the first human transmissible spongiform encephalopathy (TSE) in which a mutation in a gene encoding for prion protein (PrP) was discovered. Its true prevalence is difficult to estimate but figures within the range of 1-10/100,000,000 are quoted. GSS is defined as a neurodegenerative...

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PMID: 16903147

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GSS

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