Molecular pathogenesis of sporadic prion diseases in man.

摘要: The yeast, fungal and mammalian prions determine heritable infectious traits that are encoded in alternative conformations of proteins. They cause lethal sporadic, familial neurodegenerative conditions man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome (GSS), kuru, sporadic fatal insomnia (SFI) likely variable protease-sensitive prionopathy (VPSPr). most prevalent human prion diseases is (s)CJD. Recent advances amplification detection led to considerable optimism early possibly preclinical diagnosis therapy might become a reality. Although several drugs have already been tested small numbers sCJD patients, there no clear evidence any agent's efficacy. Therefore, it remains crucial the full spectrum strains conformational features pathogenic protein governing replication prions. Research this direction essential for rational development diagnostic as well therapeutic strategies. Moreover, growing recognition fundamental processes involved propagation - intercellular induction misfolding seeded aggregation misfolded host proteins far wider significance. This insight leads new avenues research ever-widening age-related caused by pose major challenge healthcare.