[HTML][HTML] The biological function of the cellular prion protein: an update
MA Wulf, A Senatore, A Aguzzi - BMC biology, 2017 - Springer
The misfolding of the cellular prion protein (PrP C ) causes fatal neurodegenerative diseases.
Yet PrP C is highly conserved in mammals, suggesting that it exerts beneficial functions …
Yet PrP C is highly conserved in mammals, suggesting that it exerts beneficial functions …
The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6
…, C Doucerain, R Marpakwar, P Bakirci, A Senatore… - Nature, 2016 - nature.com
Ablation of the cellular prion protein PrP C leads to a chronic demyelinating polyneuropathy
affecting Schwann cells. Neuron-restricted expression of PrP C prevents the disease 1 , …
affecting Schwann cells. Neuron-restricted expression of PrP C prevents the disease 1 , …
[PDF][PDF] Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model
A familial form of Creutzfeldt-Jakob disease (CJD) is linked to the D178N/V129 prion protein
(PrP) mutation. Tg(CJD) mice expressing the mouse homolog of this mutant PrP synthesize …
(PrP) mutation. Tg(CJD) mice expressing the mouse homolog of this mutant PrP synthesize …
[PDF][PDF] Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α2δ-1 subunit
A Senatore, S Colleoni, C Verderio, E Restelli, R Morini… - Neuron, 2012 - cell.com
How mutant prion protein (PrP) leads to neurological dysfunction in genetic prion diseases
is unknown. Tg(PG14) mice synthesize a misfolded mutant PrP which is partially retained in …
is unknown. Tg(PG14) mice synthesize a misfolded mutant PrP which is partially retained in …
[HTML][HTML] The epidemiology of persons living with Fontan in 2020 and projections for 2030: development of an epidemiology model providing multinational estimates
L Plappert, S Edwards, A Senatore, A De Martini - Advances in Therapy, 2022 - Springer
Introduction Fontan surgery is a palliative procedure performed in children with a functionally
univentricular heart. Improvements in surgical technique over the past 30 years have …
univentricular heart. Improvements in surgical technique over the past 30 years have …
[HTML][HTML] Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity
…, AN Shrivastava, P Bakirci, S Sorce, A Senatore… - PLoS …, 2017 - journals.plos.org
Prion infections cause inexorable, progressive neurological dysfunction and neurodegeneration.
Expression of the cellular prion protein PrP C is required for toxicity, suggesting the …
Expression of the cellular prion protein PrP C is required for toxicity, suggesting the …
[HTML][HTML] A conformational switch controlling the toxicity of the prion protein
K Frontzek, M Bardelli, A Senatore, A Henzi… - Nature Structural & …, 2022 - nature.com
Prion infections cause conformational changes of the cellular prion protein (PrP C ) and
lead to progressive neurological impairment. Here we show that toxic, prion-mimetic ligands …
lead to progressive neurological impairment. Here we show that toxic, prion-mimetic ligands …
Glial activation in prion diseases is selectively triggered by neuronal PrPSc
AKK Lakkaraju, S Sorce, A Senatore… - Brain …, 2022 - Wiley Online Library
Although prion infections cause cognitive impairment and neuronal death, transcriptional
and translational profiling shows progressive derangement within glia but surprisingly little …
and translational profiling shows progressive derangement within glia but surprisingly little …
A mutant prion protein sensitizes neurons to glutamate-induced excitotoxicity
…, IH Solomon, T Massignan, A Senatore… - Journal of …, 2013 - Soc Neuroscience
Growing evidence suggests that a physiological activity of the cellular prion protein (PrP C )
plays a crucial role in several neurodegenerative disorders, including prion and Alzheimer's …
plays a crucial role in several neurodegenerative disorders, including prion and Alzheimer's …
Non‐infectious aggregates of the prion protein react with several PrPSc‐directed antibodies
…, HM Christensen, A Senatore… - Journal of …, 2008 - Wiley Online Library
The key event in the pathogenesis of prion diseases is the conformational conversion of the
normal prion protein (PrP) (PrP C ) into an infectious, aggregated isoform (PrP Sc ) that has …
normal prion protein (PrP) (PrP C ) into an infectious, aggregated isoform (PrP Sc ) that has …