Mutations in prion protein gene: pathogenic mechanisms in C-terminal vs. N-terminal domain, a review

L Bernardi, AC Bruni - International Journal of Molecular Sciences, 2019 - mdpi.com
Inherited mutations in the Prion protein (PrP), encoded by the PRNP gene, have been
associated with autosomal dominant neurodegenerative disorders, such as Creutzfeldt …

PrPC signalling in neurons: from basics to clinical challenges

TZ Hirsch, J Hernandez-Rapp, S Martin-Lannerée… - Biochimie, 2014 - Elsevier
The cellular prion protein PrP C was identified over twenty-five years ago as the normal
counterpart of the scrapie prion protein PrP Sc, itself the main if not the sole component of …

Permselectivity of Silk Fibroin Hydrogels for Advanced Drug Delivery Neurotherapies

R Fernández-Serra, A Lekouaghet, L Peracho… - …, 2024 - ACS Publications
A promising trend in tissue engineering is using biomaterials to improve the control of drug
concentration in targeted tissue. These vehicular systems are of specific interest when the …

Prion protein facilitates synaptic vesicle release by enhancing release probability

SW Robinson, ML Nugent, D Dinsdale… - Human molecular …, 2014 - academic.oup.com
The cellular prion protein (PrPC) has been implicated in several neurodegenerative
diseases as a result of protein misfolding. In humans, prion disease occurs typically with a …

An insight of early PrP‐E200K aggregation by combined molecular dynamics/fragment molecular orbital approaches

R Paciotti, L Storchi, A Marrone - Proteins: Structure, Function …, 2019 - Wiley Online Library
Unveiling the events leading to the formation of prion particles is a nowadays challenge in
the field of neurochemistry. Pathogenic mutants of prion protein (PrP) are characterized by …

Roles of prion proteins in mammalian development

YP Cheon, C Ryou, ŽM Svedružić - Animal Cells and Systems, 2024 - Taylor & Francis
Prion protein (PrP) is highly conserved and is expressed in most tissues in a developmental
stage-specific manner. Glycosylated cellular prion protein (PrPC) is found in most cells and …

Prion protein as a mediator of synaptic transmission

JR Steinert - Communicative & Integrative Biology, 2015 - Taylor & Francis
Neurodegenerative disorders are characterized by synaptic and neuronal dysfunction which
precedes general neuronal loss and subsequent cognitive or behavioral anomalies …

Phosphorylation mapping of laminin γ1-chain: Kinases, functional interaction sequences, and phosphorylation-interfering cancer mutations

PA Galliou, KM Verrou, NA Papanikolaou… - Journal of …, 2024 - Springer
We computationally predicted all phosphorylation sites in the sequence of the human
laminin γ1-chain (LAMC1), and computationally identified, for the first time, all kinases for …

Hampering the early aggregation of PrP-E200K protein by charge-based inhibitors: a computational study

M Agamennone, L Storchi, A Marrone… - Journal of Computer …, 2021 - Springer
A multilayered computational workflow was designed to identify a druggable binding site on
the surface of the E200K pathogenic mutant of the human prion protein, and to investigate …

Loss of STI 1‐mediated neuronal survival and differentiation in disease‐associated mutations of prion protein

MC Landemberger, GP de Oliveira… - Journal of …, 2018 - Wiley Online Library
Cellular prion protein (Pr PC) is widely expressed and displays a variety of well‐described
functions in the central nervous system (CNS). Mutations of the PRNP gene are known to …