Distinct synthetic Aβ prion strains producing different amyloid deposits in bigenic mice
Jan Stöhra,b, Carlo Condelloa, Joel C. Wattsa,b,1, Lillian Blocha, Abby Oehlerc, Mimi Nickd, Stephen J. DeArmonda,c, Kurt Gilesa,b, William F. DeGradod, and Stanley B. Prusinera,b,2 Author Affiliations
↵2To whom correspondence should be addressed. E-mail: email@example.com. Author contributions: J.S., C.C., and S.B.P. designed research; J.S., C.C., J.C.W., L.B., A.O., and M.N. performed research; J.S., C.C., S.J.D., K.G., W.F.D., and S.B.P. analyzed data; and J.S. and S.B.P. wrote the paper.
The authors declare no conflict of interest.
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Prions causing neurodegeneration: A unifying etiology and the quest for therapeutics
Stanley B Prusiner University of California, Institute for Neurodegenerative Diseases; San Francisco, CA, USA
Mounting evidence argues that prions feature in the pathogenesis of many, if not all, neurodegenerative diseases. Such disorders include Alzheimer’s, Parkinson’s, Lou Gehrig’s and Creutzfeldt-Jakob diseases as well as the frontotemporal dementias. In each of these illnesses, aberrant forms of a particular protein accumulate as pathological deposits referred to as amyloid plaques, neurofibrillary tangles, Lewy bodies, as well as glial cytoplasmic and/or nuclear inclusions. The heritable forms of the neurodegenerative diseases are often caused by mutations in the genes encoding the mutant, prion proteins that accumulate in the CNS of patients with these fatal disorders. The late onset of the inherited neurodegenerative diseases seems likely to be explained by the protein quality control systems being less efficient in older neurons and thus, more permissive for prion accumulation. To date, there is not a single drug that halts or even slows one neurodegenerative disease.
Prusiner SB. Biology and genetics of prions causing neurodegeneration. Annu Rev Genet 2013; 47:601-23; PMID:24274755; http://dx.doi.org/10.1146/ annurev-genet-110711-155524
Jucker M, Walker LC. Self-propagation of pathogenic protein aggregates in neurodegenerative diseases. Nature 2013; 501:45-51; PMID:24005412; http://dx.doi. org/10.1038/nature12481
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