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Science 6 February 1998:
Vol. 279. no. 5352, pp. 827 - 834
DOI: 10.1126/science.279.5352.827
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Research Articles

A Transmembrane Form of the Prion Protein in Neurodegenerative Disease

Ramanujan S. Hegde, James A. Mastrianni, Michael R. Scott, Kathryn A. DeFea, Patrick Tremblay, Marilyn Torchia, Stephen J. DeArmond, Stanley B. Prusiner, Vishwanath R. Lingappa *

At the endoplasmic reticulum membrane, the prion protein (PrP) can be synthesized in several topological forms. The role of these different forms was explored with transgenic mice expressing PrP mutations that alter the relative ratios of the topological forms. Expression of a particular transmembrane form (termed CtmPrP) produced neurodegenerative changes in mice similar to those of some genetic prion diseases. Brains from these mice contained CtmPrP but not PrPSc, the PrP isoform responsible for transmission of prion diseases. Furthermore, in one heritable prion disease of humans, brain tissue contained CtmPrP but not PrPSc. Thus, aberrant regulation of protein biogenesis and topology at the endoplasmic reticulum can result in neurodegeneration.

R. S. Hegde and K. A. DeFea are in the Department of Physiology, University of California, San Francisco, CA 94143-0444, USA. J. A. Mastrianni, M. R. Scott, P. Tremblay, and M. Torchia are in the Department of Neurology, University of California, San Francisco, CA 94143-0518, USA. S. J. DeArmond is in the Departments of Pathology and Neurology, University of California, San Francisco, CA 94143, USA. S. B. Prusiner is in the Departments of Neurology and Biochemistry and Biophysics, University of California, San Francisco, CA 94143-0518, USA. V. R. Lingappa is in the Departments of Physiology and Medicine, University of California, San Francisco, CA 94143, USA.
*   To whom correspondence should be addressed. E-mail: vrl{at}itsa.ucsf.edu

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Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease.
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A 7-kDa Prion Protein (PrP) Fragment, an Integral Component of the PrP Region Required for Infectivity, Is the Major Amyloid Protein in Gerstmann-Straussler-Scheinker Disease A117V.
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