期刊
JOURNAL OF MOLECULAR NEUROSCIENCE
卷 45, 期 3, 页码 422-424出版社
HUMANA PRESS INC
DOI: 10.1007/s12031-011-9543-1
关键词
Variably protease-sensitive prionopathy; Prions; Prion protein; Prion diseases; Creutzfeldt-Jakob disease; Alzheimer's disease
资金
- National Institutes of Health (NIH) [AG14359, AG08702, R01NS062787]
- Centers for Disease Control and Prevention [CCU 515004]
- Britton Fund
- CJD Foundation, Alliance BioSecure
- University Center on Aging and Health
- McGregor Foundation
- Case Western Reserve University
Variably protease-sensitive prionopathy (VPSPr) is a novel disease involving the prion protein (PrP) that has clinical similarities with non-Alzheimer's dementias especially frontotemporal dementia, diffuse Lewis body disease, and normal pressure hydrocephalus. VPSPr can be distinguished from sporadic Creutzfeldt-Jakob disease (sCJD) especially for the characteristics of the abnormal PrP. Furthermore, although VPSPr like sCJD affects patients with the three PrP genotypes as determined by the common methionine/valine polymorphism, the allelic prevalence is very different in the two diseases. These findings suggest that VPSPr is basically different from classical prion diseases such as sCJD being perhaps more akin to other neurodegenerative dementias.
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