Prion strains viewed through the lens of cryo-EM

SW Manka, A Wenborn, J Collinge… - Cell and Tissue …, 2023 - Springer
Mammalian prions are lethal transmissible pathogens that cause fatal neurodegenerative
diseases in humans and animals. They consist of fibrils of misfolded, host-encoded prion …

Advanced situation with recombinant toxins: Diversity, production and application purposes

E Efremenko, A Aslanli, I Lyagin - International Journal of Molecular …, 2023 - mdpi.com
Today, the production and use of various samples of recombinant protein/polypeptide toxins
is known and is actively developing. This review presents state-of-the-art in research and …

[HTML][HTML] Aβ plaques

LC Walker - Free neuropathology, 2020 - ncbi.nlm.nih.gov
Aβ plaques are one of the two lesions in the brain that define the neuropathological
diagnosis of Alzheimer's disease. Plaques are highly diverse structures; many of them …

Cross-seeding by prion protein inactivates TDP-43

SA Polido, C Stuani, A Voigt, P Banik, J Kamps… - Brain, 2024 - academic.oup.com
A common pathological denominator of various neurodegenerative diseases is the
accumulation of protein aggregates. Neurotoxic effects are caused by a loss of the …

The degree of astrocyte activation is predictive of the incubation time to prion disease

N Makarava, O Mychko, JCY Chang… - Acta Neuropathologica …, 2021 - Springer
In neurodegenerative diseases including Alzheimer's, Parkinson's and prion diseases,
astrocytes acquire disease-associated reactive phenotypes. With growing appreciation of …

Non-cell autonomous astrocyte-mediated neuronal toxicity in prion diseases

R Kushwaha, A Sinha, N Makarava… - Acta Neuropathologica …, 2021 - Springer
Under normal conditions, astrocytes perform a number of important physiological functions
centered around neuronal support and synapse maintenance. In neurodegenerative …

Convergent generation of atypical prions in knockin mouse models of genetic prion disease

S Mehra, MEC Bourkas, L Kaczmarczyk, E Stuart… - The Journal of Clinical …, 2024 - jci.org
Most cases of human prion disease arise due to spontaneous misfolding of WT or mutant
prion protein, yet recapitulating this event in animal models has proven challenging. It …

Inflammation and Parkinson's disease pathogenesis: mechanisms and therapeutic insight

G Forloni, P La Vitola, M Cerovic, C Balducci - Progress in molecular …, 2021 - Elsevier
Abstract After Alzheimer's disease, Parkinson's disease is the most frequent
neurodegenerative disorder. Although numerous treatments have been developed to control …

Microglia deficiency accelerates prion disease but does not enhance prion accumulation in the brain

BM Bradford, LI McGuire, DA Hume, C Pridans… - Glia, 2022 - Wiley Online Library
Prion diseases are transmissible, neurodegenerative disorders associated with misfolding of
the prion protein. Previous studies show that reduction of microglia accelerates central …

Single-cell transcriptomics unveils molecular signatures of neuronal vulnerability in a mouse model of prion disease that overlap with Alzheimer's disease

JA Slota, L Lamoureux, KL Frost, BV Sajesh… - Nature …, 2024 - nature.com
Understanding why certain neurons are more sensitive to dysfunction and death caused by
misfolded proteins could provide therapeutically relevant insights into neurodegenerative …