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Article

Gerstmann-Sträussler-Scheinker: a new phenotype with 'curly' PrP deposits.

Journal of neuropathology and experimental neurology - 1 Jul 2006

Colucci Monica, Moleres Francisco J, Xie Zhi-Liang, Ray-Chaudhury Abhik, Gutti Sujata, Butefisch Cathrin M, Cervenakova Larisa, Wang Wen, Goldfarb Lev G, Kong Qingzhong, Ghetti Bernardino, Chen Shu G, Gambetti Pierluigi

Abstract excerpt

Gerstmann-Sträussler-Scheinker (GSS) is a hereditary prion disease typically associated with prion protein (PrP)-containing plaques. The protease-resistant, scrapie PrP (PrPSc) is represented by internal fragments, whereas the C-terminal fragments associated with the other prion diseases are generally underrepresented. Different histopathologic and PrPSc features associated with at least 13 PrP gene (PRNP)...

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Identifiers

PMID: 16825951
DOI: 10.1097/01.jnen.0000228198.81797.4d

Genes

GSS
PRNP

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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