Fabrizio Tagliavini
Carlo Besta Neurological Institute
Publications 383
Parkinson’s disease (PD) is a neurodegenerative disorder whose diagnosis is often challenging because symptoms may overlap with neurodegenerative parkinsonisms. PD is characterized by intraneuronal accumulation of abnormal α-synuclein in brainstem while neurodegenerative parkinsonisms might be associated with accumulation of either α-synuclein, as in the case of Multiple System Atrophy (MSA) or tau, as in the case of Corticobasal Degeneration (CBD) and Progressive Supranuclear Palsy (PSP), in ot...
1 CitationsSource
Prion diseases are neurodegenerative disorders which are caused by an accumulation of the abnormal, misfolded prion protein known as scrapie prion protein (PrPSc). These disorders are unique as they occur as sporadic, genetic and acquired forms. Sporadic Creutzfeldt-Jakob Disease (CJD) is the most common human prion disease, accounting for approximately 85–90% of cases, whereas autosomal dominant genetic forms, due to mutations in the prion protein gene (PRNP), account for 10–15% of cases. Genet...
1 CitationsSource
#1Ignazio Cali (Case Western Reserve University)H-Index: 16
#2Jody Lavrich (Case Western Reserve University)H-Index: 1
Last.Silvio Notari (Case Western Reserve University)H-Index: 6
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The presence of abnormal, disease-related prion protein (PrPD) has recently been demonstrated by protein misfolding cyclic amplification (PMCA) in urine of patients affected with variant Creutzfeldt-Jakob disease (vCJD), a prion disease typically acquired from consumption of prion contaminated bovine meat. The complexity and multistage process of urine excretion along with the obligatory use of PMCA raise the issue of whether strain characteristics of the PrPD present in vCJD brains, such as inf...
#1Paolo VitaliH-Index: 22
#2Fulvia PalesiH-Index: 8
Last.Michael D. Geschwind (UCSF: University of California, San Francisco)H-Index: 39
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#1Edoardo BistaffaH-Index: 4
#2Martina Rossi (SISSA: International School for Advanced Studies)H-Index: 2
Last.Fabio ModaH-Index: 14
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Prion diseases are a group of neurodegenerative disorders associated with the conformational conversion of the cellular prion protein (PrPC) into an abnormal misfolded form named PrPSc. Other than accumulating in the brain, PrPSc can bind PrPC and force it to change conformation to PrPSc. The exact mechanism which underlies the process of PrPC/PrPSc conversion still needs to be defined and many molecules or cofactors might be involved. Several studies have documented an important role of PrPC to...
1 CitationsSource
#1Tamara P. TavaresH-Index: 4
Last.Elizabeth FingerH-Index: 25
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Objective To characterize the time course of ventricular volume expansion in genetic frontotemporal dementia (FTD) and identify the onset time and rates of ventricular expansion in presymptomatic FTD mutation carriers. Methods Participants included patients with a mutation in MAPT, PGRN, or C9orf72, or first-degree relatives of mutation carriers from the GENFI study with MRI scans at study baseline and at 1 year follow-up. Ventricular volumes were obtained from MRI scans using FreeSurfer, with m...
1 CitationsSource
#1Paolo Maria Rossini (CUA: The Catholic University of America)H-Index: 80
#2Stefano F. CappaH-Index: 72
Last.Nicola VanacoreH-Index: 34
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#1Stefano Gazzina (University of Brescia)H-Index: 10
#2Mario Grassi (UNIPV: University of Pavia)H-Index: 30
Last.Barbara Borroni (University of Brescia)H-Index: 48
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Objective Cognitively engaging lifestyles have been associated with reduced risk of conversion to dementia. Multiple mechanisms have been advocated, including increased brain volumes (ie, brain reserve) and reduced disease progression (ie, brain maintenance). In cross-sectional studies of presymptomatic frontotemporal dementia (FTD), higher education has been related to increased grey matter volume. Here, we examine the effect of education on grey matter loss over time. Methods Two-hundred twent...
#1Elisa Meneghetti (SISSA: International School for Advanced Studies)H-Index: 2
#2Lisa Gasperini (SISSA: International School for Advanced Studies)H-Index: 4
Last.Giuseppe Legname (SISSA: International School for Advanced Studies)H-Index: 39
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Prion diseases are fatal neurodegenerative disorders characterized by the cellular prion protein (PrPC) conversion into a misfolded and infectious isoform termed prion or PrPSc. The neuropathological mechanism underlying prion toxicity is still unclear, and the debate on prion protein gain- or loss-of-function is still open. PrPC participates to a plethora of physiological mechanisms. For instance, PrPC and copper cooperatively modulate N-methyl-D-aspartate receptor (NMDAR) activity by mediating...
1 CitationsSource