Article
Aggregation of prion protein with insertion mutations is proportional to the number of inserts.
The Biochemical journal - 15 Apr 2007
Yu Shuiliang, Yin Shaoman, Li Chaoyang, Wong Poki, Chang Binggong, Xiao Fan, Kang Shin-Chung, Yan Huimin, Xiao Gengfu, Tien Po, Sy Man-Sun
Abstract excerpt
Mutation in the prion gene, PRNP, accounts for approx. 10-15% of human prion diseases. However, little is known about the mechanisms by which a mutant prion protein (PrP) causes disease. We compared the biochemical properties of a wild-type human prion protein, rPrP(C) (recombinant wild-type PrP), which has five octapeptide-repeats, with two recombinant human prion proteins with insertion mutations, one with...
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