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Previous Volume 340:1630-1638 May 27, 1999 Number 21 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text PDF Editorial by Gambetti, P. Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

The human prion diseases include Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker disease, fatal familial insomnia, and the recently described new variant of Creutzfeldt–Jakob disease. Much evidence argues that a post-translational, noncovalent modification of prion protein is the fundamental event in the mechanism underlying these diseases.¹ The normal cellular isoform of the prion protein (PrP^C) is predominantly -helical, is detergent soluble, and is readily digested by proteases. In contrast, the pathogenic isoform (PrP^Sc) has a substantially ß-sheet structure, is insoluble in nondenaturing detergents, and shows relative resistance to proteolytic digestion.^2,3,4 The protease-resistant core of PrP^Sc, designated PrP27–30, is usually detectable in . . . [Full Text of this Article]

Case Report

Methods

Genetic Analysis

Neuropathological Analysis

Protein Analysis

Immunoblotting and Histologic Examination of Transgenic Animals

Results

Neuropathological Findings

Genotyping

PrP^Sc Distribution and Conformation

Transmission Studies

Discussion

Source Information

From the Institute for Neurodegenerative Diseases, Department of Neurology (J.A.M., R.N., R.L., G.C.T., D.H., S.J.D., S.B.P.), and the Departments of Pathology (S.J.D.) and Biochemistry and Biophysics (S.B.P.), University of California, San Francisco; and the Department of Neurology, University of Chicago, Chicago (J.A.M.).

Address reprint requests to Dr. Prusiner at the Department of Neurology, Box 0518, University of California, San Francisco, CA 94143-0518.

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