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Previous Volume 344:1516-1526 May 17, 2001 Number 20 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 Roos, R. P. Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Twenty-five years ago, little was known about the causes of neurodegenerative diseases. Now, however, it is clear that they result from abnormalities in the processing of proteins. In each of these diseases, defective processing causes the accumulation of one or more specific neuronal proteins.

Of all the laboratory research on neurodegenerative diseases, the studies that led to the discovery of prions have yielded the most unexpected findings. The idea that a protein can act as an infectious pathogen and cause degeneration of the central nervous system was accepted only after a long and arduous battle.¹ The concept of prions not . . . [Full Text of this Article]

Prions

Prion Diseases

Epidemiologic Features

Neuropathological Features

Strains of Prions

Sporadic, Genetic, and Infectious Forms of Prion Disease

Other Neurodegenerative Diseases

Alzheimer's Disease

Frontotemporal Dementia and Pick's Disease

Parkinson's Disease

Amyotrophic Lateral Sclerosis

Huntington's Disease and Spinocerebellar Ataxias

Transgenic Mouse Models

Diagnostic Tests

Prevention and Treatment

Preventing Abnormal Processing of Proteins and Enhancing Their Clearance

Replacement Therapy

Speculation on the Spectrum of Degenerative Diseases

The Future

Source Information

Presented as the 110th Shattuck Lecture to the Annual Meeting of the Massachusetts Medical Society, Boston, May 20, 2000.From the Institute for Neurodegenerative Diseases and the Departments of Neurology and of Biochemistry and Biophysics, University of California, San Francisco.

Address reprint requests to Dr. Prusiner at the Institute for Neurodegenerative Diseases, Box 0518, University of California, San Francisco, CA 94143-0518.

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