Distinctive properties of the prion protein in the brain and retina in the amyloidosis associated with the PRNP F198S Mutation. *
This study provides the first comparative neuropathologic and biochemical analysis of PrP deposits in the brain and retina of individuals with GSS-associated F198S mutation, revealing that while both tissues contain seeding-capable aggregates, the retina lacks the constitutive 8 kDa proteolytic fragments found in the brain and exhibits distinct amyloid and glycosylation properties.