volume 5, issue 3, P155-173 2013
DOI: 10.18632/aging.100543
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Xiangzhu Xiao, Ignazio Cali, Zhiqian Dong, Gianfranco Puoti, Jue Yuan, Liuting Qing, Heming Wang, Qingzhong Kong, Pierluigi Gambetti, Wen-Quan Zou

Abstract: Insertion of 144-base pair (bp) containing six extra octapeptide repeats between residues 51 and 91 of prion protein (PrP) gene is associated with inherited prion diseases. Most cases linked to this insertion examined by Western blotting showed detectable proteinase K-resistant PrPSc (rPrPSc) resembling PrPSc type 1 and type 2 in sporadic Creutzfeldt-Jakob disease (sCJD), or PrP7-8 in Gerstmann-Sträussler-Scheinker disease. However, cases lacking detectable rPrPSc also have been reported. Which PrP conformer i…

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