Article
Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele.
Cell - 18 Nov 1994
Tagliavini F, Prelli F, Porro M, Rossi G, Giaccone G, Farlow M R, Dlouhy S R, Ghetti B, Bugiani O, Frangione B
Abstract excerpt
Gerstmann-Sträussler-Scheinker (GSS) disease is a cerebral amyloidosis linked to mutations of the PRNP gene. We previously reported that the amyloid protein in the Indiana kindred of GSS is an internal fragment of prion protein (PrP). To investigate whether this fragment originates only from mutant or from both mutant and wild-type PrP, we have characterized amyloid proteins purified from patients of the Indiana...
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