Article
The number of octapeptide repeat affects the expression and conversion of prion protein.
Biochemical and biophysical research communications - 15 May 2009
Hiraga Chihiro, Kobayashi Atsushi, Kitamoto Tetsuyuki
Abstract excerpt
The human prion protein (PrP) has five copies of an octapeptide repeat (OR). The mutant PrP with 6-14 OR causes the genetic form of Creutzfeldt-Jakob disease (CJD). To determine the influence of OR on the conversion of PrP, we examined the conversion efficiency of mouse mutant PrP molecules with 1-16 OR in scrapie-infected cells. The expression level of mutant PrP and the glycoform ratio of the abnormal isoform...
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