Protein misfolding and prion diseases.
The prion diseases provide an intriguing connection between protein folding and neurodegenerative disease. In this review, I explore that importance of protein folding and misfolding in the prion diseases. Thermodynamic and kinetic models are examined in an effort to understand infectious, inherited and sporadic forms of these diseases. These concepts can be generalized to gain insight into other disorders of protein aggregation and deposition such as Alzheimer's disease.
Cohen
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Departments of Cellular & Molecular Pharmacology & Medicine, University of California, San Francisco, CA 94143-0450, USA. cohen@cmpharm.ucsf.edu
D000818:Animals; D006801:Humans; D007700:Kinetics; D017096:Prion Diseases / Q000235:genetics / Q000378:metabolism*; D011328:Prions / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D017510:Protein Folding; D017433:Protein Structure, Secondary; D013816:Thermodynamics
D011328:Prions