Fabrizio Tagliavini

TL;DR: Investigation of longitudinal change in WMH and the associations of WMH burden with grey matter (GM) loss, markers of neurodegeneration and cognitive function in GRN mutation carriers revealed that WMH load was higher in both symptomatic and presymptomatic groups compared with controls and this load increased over time.

TL;DR: Hemoglobin mRNA levels in brains of patients affected by variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease, and in different genetic forms of prion diseases, suggest that gene expression of HBB and HBA1/2 in brain tissue is differentially affected by distinct prion and prion-like aggregating protein strains.

TL;DR: This work employed the PrP(Sc)-specific antibody 89-112 PrP motif-grafted IgG on mildly fixed, untreated brain sections from several cases of human prion diseases and confirmed specific binding of IgG 89- 112 to a structural determinant found exclusively on native disease-associated PrP conformations and lost following tissue denaturation or cross-linking fixation.

TL;DR: These simple-to-use and reproducible visual rating scales may be useful tools in the clinical setting for the discrimination of different mutations of frontotemporal dementia, and they may even help to identify atrophy prior to onset in those with MAPT mutations.

TL;DR: The results indicate that the molecular interactions mediating the aggregation state of PrPSc, possibly enciphering strain diversity, are differently targeted by GdnHCl, temperature, and proteases, and suggest that thermal and chemical denaturation perturb the structure of prion protein aggregates differently.