Reversal of misfolding: prion disease behavioral and physiological impairments recover following postnatal neuronal deletion of the PrP gene.
The prionoses are fatal neurodegenerative diseases caused by a pathogenic protein, PrP scrapie, that derives from misfolding of a normal form, PrP(c). These diseases progress through stages. A new study by Mallucci et al. in this issue of Neuron shows that prion disease may be reversed in mice by selective removal of the gene in neurons after early physiological, cognitive, and pathological features have developed.
Federoff
Howard J
HJ
Department of Neurology, Center for Aging and Developmental Biology, Aab Institute for Biomedical Research, University of Rochester School of Medicine and Dentistry, Box 645, 601 Elmwood Avenue, Rochester, NY 14642, USA. howard_federoff@urmc.rochester.edu
Mhyre
Timothy R
TR
D000818:Animals; D017353:Gene Deletion; D051379:Mice; D009474:Neurons / Q000502:physiology*; D017096:Prion Diseases / Q000473:pathology / Q000523:psychology* / Q000628:therapy*; D011328:Prions / Q000235:genetics*; D017510:Protein Folding
D011328:Prions