Prions, prionoids and protein misfolding disorders

C Scheckel, A Aguzzi - Nature Reviews Genetics, 2018 - nature.com
Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

[HTML][HTML] Diverse functions of the prion protein–Does proteolytic processing hold the key?

L Linsenmeier, HC Altmeppen, S Wetzel… - … et Biophysica Acta (BBA …, 2017 - Elsevier
Proteolytic processing of the cellular and disease-associated form of the prion protein leads
to generation of bioactive soluble prion protein fragments and modifies the structure and …

Exosomal cellular prion protein drives fibrillization of amyloid beta and counteracts amyloid beta‐mediated neurotoxicity

C Falker, A Hartmann, I Guett, F Dohler… - Journal of …, 2016 - Wiley Online Library
Alzheimer's disease is a common neurodegenerative, progressive, and fatal disorder.
Generation and deposition of amyloid beta (Aβ) peptides associate with its pathogenesis …

The transient receptor potential melastatin 2 (TRPM2) channel contributes to β-amyloid oligomer-related neurotoxicity and memory impairment

VG Ostapchenko, M Chen, MS Guzman… - Journal of …, 2015 - Soc Neuroscience
In Alzheimer's disease, accumulation of soluble oligomers of β-amyloid peptide is known to
be highly toxic, causing disturbances in synaptic activity and neuronal death. Multiple …

Metabotropic glutamate receptor 5 knockout reduces cognitive impairment and pathogenesis in a mouse model of Alzheimer's disease

A Hamilton, JL Esseltine, RA DeVries, SP Cregan… - Molecular brain, 2014 - Springer
Background Alzheimer's disease (AD) pathology occurs in part as the result of excessive
production of β-amyloid (Aβ). Metabotropic glutamate receptor 5 (mGluR5) is now …

Transcriptome changes in the Alzheimer's disease middle temporal gyrus: importance of RNA metabolism and mitochondria-associated membrane genes

IS Piras, J Krate, E Delvaux, J Nolz… - Journal of …, 2019 - content.iospress.com
We used Illumina Human HT-12 v4 arrays to compare RNA expression of middle temporal
gyrus (MTG; BA21) in Alzheimer's disease (AD= 97) and non-demented controls (ND= 98). A …

The sheddase ADAM10 is a potent modulator of prion disease

HC Altmeppen, J Prox, S Krasemann, B Puig… - Elife, 2015 - elifesciences.org
The prion protein (PrPC) is highly expressed in the nervous system and critically involved in
prion diseases where it misfolds into pathogenic PrPSc. Moreover, it has been suggested as …

Human amyloid β peptide and tau co-expression impairs behavior and causes specific gene expression changes in Caenorhabditis elegans

C Wang, V Saar, KL Leung, L Chen, G Wong - Neurobiology of Disease, 2018 - Elsevier
Alzheimer's disease (AD) is a progressive neurodegenerative disorder characterized by the
presence of extracellular amyloid plaques consisting of Amyloid-β peptide (Aβ) aggregates …

Activation of mGluR1 mediates C1q-dependent microglial phagocytosis of glutamatergic synapses in Alzheimer's rodent models

B Bie, J Wu, JF Foss, M Naguib - Molecular neurobiology, 2019 - Springer
Microglia and complements appear to be involved in the synaptic and cognitive deficits in
Alzheimer's disease (AD), though the mechanisms remain elusive. In this study, utilizing two …

The prion protein ligand, stress-inducible phosphoprotein 1, regulates amyloid-β oligomer toxicity

VG Ostapchenko, FH Beraldo… - Journal of …, 2013 - Soc Neuroscience
In Alzheimer's disease (AD), soluble amyloid-β oligomers (AβOs) trigger neurotoxic
signaling, at least partially, via the cellular prion protein (PrPC). However, it is unknown …