In:
Science, American Association for the Advancement of Science (AAAS), Vol. 336, No. 6088 ( 2012-06-22), p. 1511-1513
Abstract:
Many neurodegenerative diseases—including Creutzfeldt-Jakob disease, Alzheimer's disease (AD), Parkinson's disease, and amyotrophic lateral sclerosis (ALS)—share two remarkable characteristics. The first is that more than 80% of cases are sporadic. The second is that although many of the disease-specific mutant proteins are expressed in embryogenesis, the inherited forms of these neurodegenerative diseases are late-onset. This suggests that some event occurs with aging that renders a disease-specific protein pathogenic. More than 20 years ago, I argued that this event involves a stochastic refolding of the etiologic protein into a misfolded infectious state known as a prion. In the past decade, there has been renewed interest in the possibility that the proteins causing neurodegeneration are all prions, which would profoundly influence the development of diagnostics and effective therapies.
Type of Medium:
Online Resource
ISSN:
0036-8075
,
1095-9203
DOI:
10.1126/science.1222951
Language:
English
Publisher:
American Association for the Advancement of Science (AAAS)
Publication Date:
2012
detail.hit.zdb_id:
128410-1
detail.hit.zdb_id:
2066996-3
detail.hit.zdb_id:
2060783-0
SSG:
11
