Prion diseases are fatal neurodegenerative disorders caused by an aberrant accumulation of the misfolded cellular prion protein (PrPC) conformer, denoted as infectious scrapie isoform or PrPSc. Our understanding of the mechanisms by which mutations cause disease remains limited. In this work results of recent high-resolution NMR structural studies on human prion protein variants carrying pathological mutations are presented.
NMR studies of human prion proteins with inherited mutations / Biljan, I; Ilc, G; Giachin, G; Legname, Giuseppe; Plavec, J.. - (2011). (Intervento presentato al convegno SISSA/ELETTRA PRION research workshop tenutosi a Trieste).
NMR studies of human prion proteins with inherited mutations
Legname, Giuseppe;
2011-01-01
Abstract
Prion diseases are fatal neurodegenerative disorders caused by an aberrant accumulation of the misfolded cellular prion protein (PrPC) conformer, denoted as infectious scrapie isoform or PrPSc. Our understanding of the mechanisms by which mutations cause disease remains limited. In this work results of recent high-resolution NMR structural studies on human prion protein variants carrying pathological mutations are presented.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
