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Pathologic conformations of prion proteins

F E Cohen1, S B Prusiner

  • 1Department of Biochemistry and Biophysics, University of California, San Francisco 94143, USA. cohen@cgl.ucsf.edu

Annual Review of Biochemistry
|October 6, 1998
PubMed
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Prion diseases present a paradox with inherited, sporadic, and infectious forms. This review examines prion pathogenesis, exploring how an inherited disease model explains all three forms, supported by extensive experimental data.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Prion diseases exhibit unusual characteristics, notably the co-existence of inherited, sporadic, and infectious forms.
  • Understanding the molecular mechanisms of prion propagation is crucial to reconcile these diverse disease presentations.

Purpose of the Study:

  • To review prion disease pathogenesis by examining a unifying molecular mechanism.
  • To explore how an autosomal dominant inherited disease model can explain sporadic and infectious prion disease forms.
  • To analyze phenomenological constraints on prion replication models using biophysical data.

Main Methods:

  • Review of existing literature on prion disease biology.
  • Analysis of histopathological, biochemical, biophysical, and genetic studies.

Related Experiment Videos

  • Examination of transgenetic data and prion protein structural studies.

    • Main Results:

    • The review proposes a framework where an intrinsically inherited disease can manifest in sporadic and infectious forms.
    • Biophysical studies of prion protein structures provide critical constraints for replication models.
    • Experimental data from multiple disciplines support the proposed pathogenesis model.

    Conclusions:

    • A unified molecular mechanism can explain the coexistence of inherited, sporadic, and infectious prion diseases.
    • Further research integrating biophysical and genetic data is essential for a complete understanding of prion replication.
    • This review provides a comprehensive perspective on prion disease pathogenesis.