The cellular form of the prion protein is involved in controlling cell cycle dynamics, self‐renewal, and the fate of human embryonic stem cell differentiation [PDF]
, 2012 Young Jin Lee, Ilia V. Baskakov
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Brain Pathology, EarlyView.The efficacy of the novel anti‐tau active immunotherapy, p5555kb, was tested using two mouse models of tau pathology. p5555kb inoculation increased the survival rate and reduced tau pathology in tau‐overexpressing P301L mice and decreased tau seeding in the brains of C57BL/6 mice injected with human‐purified Alzheimer's disease tau.
Christopher M. Brown +7 more
wiley +1 more source
On the statistical mechanics of prion diseases
, 2001 We simulate a two-dimensional, lattice based, protein-level statistical mechanical model for prion diseases (e.g., Mad Cow disease) with concommitant prion protein misfolding and aggregation.
A. Coghlan +25 more
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The prion 2018 round tables (I): the structure of PrPSc [PDF]
, 2019 Ilia V. Baskakov +5 more
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Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice
Brain Pathology, EarlyView.We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga +11 more
wiley +1 more source
Prion-induced neurotoxicity: Possible role for cell cycle activity and DNA damage response. [PDF]
, 2015 Protein misfolding neurodegenerative diseases arise through neurotoxicity induced by aggregation of host proteins. These conditions include Alzheimer's disease, Huntington's disease, Parkinson's disease, motor neuron disease, tauopathies and prion ...
core +1 more source
Liquid–Liquid Phase Separation in Major Hallmarks of Cancer
Cell Proliferation, EarlyView.Aberrant condensates formed through phase separation are involved in the dysregulation of various critical cellular processes, including genome stability, transcriptional regulation and signal transduction, thereby promoting malignant transformation and the acquisition of multiple cancer hallmarks.
Chen‐chen Xie +10 more
wiley +1 more source
Epidemiology of progressive intellectual and neurological deterioration in UK children
Developmental Medicine &Child Neurology, EarlyView.This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity +3 more
wiley +1 more source
Development and usefulness of new polymerase chain reaction-based tests for detection of different alleles at codons 136 and 171 of the ovine prion protein gene [PDF]
, 1999 Vilma Yuzbasiyan‐Gurkan +3 more
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Protein tandem repeats that produce frameshifts can generate new structural states and functions
The FEBS Journal, EarlyView.We explored an alternative protein structure landscape by analyzing amino acid sequences from frameshifted tandem repeats—regions prone to frameshifts. These frameshifts, especially in short repeats, lead to more drastic changes than in non‐repetitive regions, often altering structure, function, localization, and potentially contributing to disease ...
Zarifa Osmanli +8 more
wiley +1 more source