abstract |
Compounds, methods and pharmaceutical compositions are provided for reducing the amount or activity of PRNP RNA in a cell or animal, and in certain instances, reducing the amount of PrP protein in a cell or animal. The compounds, methods and pharmaceutical compositions are useful for ameliorating at least one symptom or characteristic of a neurodegenerative disease. Such symptoms or features are spongy changes in the brain, development of abnormal protein aggregates, neuronal loss, markers of neuronal loss, rapidly progressing dementia and death. The neurodegenerative disease is prion disease, Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD), familial Creutzfeldt-Jakob disease (fCJD), Gerstmann-Stroisler-Scheinker syndrome, fatal familial sexual insomnia, kuru, Alzheimer's disease or Parkinson's disease. |