
Stem Cell Reports, Journal Year: 2024, Volume and Issue: unknown
Published: Sept. 1, 2024
Language: Английский
Stem Cell Reports, Journal Year: 2024, Volume and Issue: unknown
Published: Sept. 1, 2024
Language: Английский
Neurobiology of Disease, Journal Year: 2025, Volume and Issue: unknown, P. 106802 - 106802
Published: Jan. 1, 2025
Bank voles are susceptible to prion strains from many different species, yet the molecular mechanisms underlying ability of bank vole protein (BVPrP) function as a universal acceptor remain unclear. Potential differences in environments and interaction networks on cell surface brain cells may contribute BVPrP's unusual behavior. To test this hypothesis, we generated knock-in mice that express physiological levels BVPrP (M109 isoform) employed mass spectrometry compare interactomes mouse (Mo) PrP following mild vivo crosslinking tissue. Substantial overlap was observed between top interactors for MoPrP, with established PrP-interactors such neural adhesion molecules, subunits Na
Language: Английский
Citations
1Journal of Clinical Investigation, Journal Year: 2024, Volume and Issue: 134(15)
Published: July 31, 2024
Most cases of human prion disease arise due to spontaneous misfolding WT or mutant protein, yet recapitulating this event in animal models has proven challenging. It remains unclear whether generation can occur within the mouse lifespan absence protein overexpression and how disease-causing mutations affect strain properties. To address these issues, we generated knockin mice that express misfolding-prone bank vole (BVPrP). While expressing BVPrP (I109 variant) remained free from neurological disease, a subset with (D178N E200K) causing genetic developed progressive illness. Brains spontaneously ill contained disease-specific neuropathological changes as well atypical protease-resistant BVPrP. Moreover, brain extracts D178N- E200K-mutant BVPrP-knockin exhibited seeding activity transmitted Surprisingly, properties prions appeared identical before after transmission, suggesting both guide formation similar strain. These findings imply develop bona fide diseases may share uniform initial mechanism action.
Language: Английский
Citations
6Ageing Research Reviews, Journal Year: 2025, Volume and Issue: unknown, P. 102667 - 102667
Published: Jan. 1, 2025
Language: Английский
Citations
0Stem Cell Reports, Journal Year: 2024, Volume and Issue: unknown
Published: Sept. 1, 2024
Language: Английский
Citations
1