Prion disease, Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, frontotemporal dementia and amyotrophic lateral sclerosis all share a common etiology: neurodegeneration linked to the misfolding and aggregation of a specific protein. But the causes of neuronal death in these diseases are still poorly understood, impeding the development of strategies to prevent neurodegeneration. To fill this knowledgeContinueContinue reading “Protecting neurons from misfolded prion proteins”
Tag Archives: prions
Mouse models of prion diseases
Prion diseases are unusual, infectious, potentially deadly, neurodegenerative diseases caused by transmission and toxic accumulation of misshapen prion protein (PrP) in the brain. A lack of animal models that accurately recapitulate prion disease pathology has impeded our understanding in this area; therefore, the characterization of new mouse models of fatal familial insomnia (FFI) and Creutzfeld-JakobContinueContinue reading “Mouse models of prion diseases”
Prions cross the species barrier
Prions are abnormal proteins whose accumulation in brain tissues can cause disease in their hosts. Prion diseases occur in many different species, and some can be transmitted between species. For example, variant Creutzfeld-Jakob disease occurs in humans after exposure to the cow prion disease bovine spongiform encephalopathy (or mad cow disease). Generally, such inter-species transmissionContinueContinue reading “Prions cross the species barrier”
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