Adriano Aguzzi

2 months ago | cell.com | Carlo Scialo |Weijia Zhong |Somanath Jagannath |Oscar G. Wilkins |Davide Caredio |Marian Hruska-Plochan | +8 more

Keywords TDP-43 low-complexity domain RNA-binding proteins aggregation seeding spreading loss of function cryptic splicing neurodegeneration ALS/FTD Introduction Despite apparent differences, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are increasingly recognized to have clinical, genetic, and pathological overlap.1 Transactive response DNA-binding protein of 43 kDa (TDP-43) is the major component of ubiquitinated cytoplasmic inclusions observed in both ALS and FTD...

Jul 28, 2024 | nature.com | Shahar Bracha |Athena Economides |Daniel Walsh |Adriano Aguzzi |Lilach Sheiner

AbstractDelivering macromolecules across biological barriers such as the blood–brain barrier limits their application in vivo. Previous work has demonstrated that Toxoplasma gondii, a parasite that naturally travels from the human gut to the central nervous system (CNS), can deliver proteins to host cells. Here we engineered T.

Apr 28, 2024 | biorxiv.org | Adriano Aguzzi |Jiang-An Yin |David J. Vocadlo |Kathi Ging

AbstractMutations in the GBA gene, which encodes the lysosomal enzyme β-glucocerebrosidase (GCase), are the most prevalent genetic susceptibility factor for Parkinson's disease (PD). However, only approximately 20% of carriers develop the disease, suggesting the presence of genetic modifiers influencing the risk of developing PD in the presence of GBA mutations.

Mar 30, 2024 | biorxiv.org | Erica Stuart |Adriano Aguzzi |Raphaella W.L. So |Aeen Ebrahim Amini

AbstractThe cellular prion protein, PrPC, has been postulated to function as a receptor for α-synuclein, potentially facilitating cell-to-cell spreading and/or toxicity of α-synuclein aggregates in neurodegenerative disorders such as Parkinson's disease. To test this hypothesis, we compared the propagation behavior of two different α-synuclein aggregate strains in M83 transgenic mice that either expressed or did not express PrPC.

Mar 27, 2024 | nature.com | Nikita Vladimirov |Fabian Voigt |Sven Hildebrand |Dominik Groos |José María Mateos |Philipp Bethge | +5 more

AbstractIn 2015, we launched the mesoSPIM initiative, an open-source project for making light-sheet microscopy of large cleared tissues more accessible. Meanwhile, the demand for imaging larger samples at higher speed and resolution has increased, requiring major improvements in the capabilities of such microscopes.