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Altered properties of amyloidogenic prion protein in genetic Creutzfeldt-Jakob disease with PRNP V180I mutation in response to pentosan polysulfate.

Brain pathology (Zurich, Switzerland) - 1 Sept 2023

Shijo Masahiro, Yoshimura Motoi, Omae Tsuyoshi, Hashimoto Go, Mizoguchi Tadataka, Kuwashiro Takahiro, Komori Takashi, Tsuboi Yoshio, Saito Tomoko, Nakagawa Masanori, Itoh Kyoko, Honda Hiroyuki

Abstract excerpt

Genetic Creutzfeldt-Jakob disease (gCJD) with V180I prion protein gene (PRNP) mutation shows weaker prion protein (PrP) deposition histologically compared with sporadic CJD, and it is more difficult to detect protease-resistant prion protein in immunoblotting. However, we previously reported the autopsy case of a patient with V180I gCJD who was treated with pentosan polysulfate sodium (PPS); this case had...

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PMID: 37525413
DOI: 10.1111/bpa.13197
PMCID: PMC10467033

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