[HTML][HTML] Truncated forms of the human prion protein in normal brain and in prion diseases
The cellular form of the prion protein (PrP c) is a glycoprotein anchored to the cell membrane
by a glycosylphosphatidylinositol moiety. An aberrant form of PrP c that is partially resistant
to proteases, PrP res, is a hallmark of prion diseases, which in humans include Creutzfeldt-
Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial
insomnia. We have characterized the major forms of PrP in normal and pathological human
brains. A COOH-terminal fragment of PrP c, designated C1, is abundant in normal and CJD …
by a glycosylphosphatidylinositol moiety. An aberrant form of PrP c that is partially resistant
to proteases, PrP res, is a hallmark of prion diseases, which in humans include Creutzfeldt-
Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial
insomnia. We have characterized the major forms of PrP in normal and pathological human
brains. A COOH-terminal fragment of PrP c, designated C1, is abundant in normal and CJD …