Age of onset and death in inherited prion disease are heritable
β Scribed by T.E.F. Webb; J. Whittaker; J. Collinge; S. Mead
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 143 KB
- Volume
- 150B
- Category
- Article
- ISSN
- 1552-4841
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## Abstract The effect of the apolipoprotein E (APOE) Ο΅4 allele on age of onset was analyzed in two groups of families with earlyβonset Alzheimer's disease (AD), (1) Volga German (VG) kindreds, in which AD is caused by an unknown locus and (2) earlyβonset nonβVG families showing evidence of linkage
Inherited prion diseases are characterized by mutations in the PRNP gene encoding the prion protein (PrP). As the other sporadic or infectious prion disease forms, they are almost all characterized by the accumulation in the brain of an abnormal misfolded form of the patient's PrP. Brain extracts ca