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.
|Titolo:||NMR studies of human prion proteins with inherited mutations|
|Titolo del libro:||4 th SISSA/ELETTRA PRION research workshop : [Trieste, Italy], January 10th, 2011|
|Data di pubblicazione:||2011|
|Appare nelle tipologie:||4.2 Abstract of congress proceedings|