Article
Mutant PrPCJD prevails over wild-type PrPCJD in the brain of V210I and R208H genetic Creutzfeldt-Jakob disease patients.
Biochemical and biophysical research communications - 14 Nov 2014
Cardone Franco, Principe Serena, Schininà Maria Eugenia, Maras Bruno, Capellari Sabina, Parchi Piero, Notari Silvio, Di Francesco Laura, Poleggi Anna, Galeno Roberta, Vinci Ramona, Mellina Vittorio, Almonti Susanna, Ladogana Anna, Pocchiari Maurizio
Abstract excerpt
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder characterized by the deposition of the pathological conformer (PrP(CJD)) of the host encoded cellular prion protein (PrP(C)). In genetic CJD associated with V210I or R208H PrP substitutions, the pathogenic role of mutant residues is still poorly understood. To understand how V210I or R208H PrP mutations facilitate the development of the disease, we...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.