A5091430110- Amyotrophic Lateral Sclerosis Research
- RNA Research and Splicing
- Prion Diseases and Protein Misfolding
- Neurogenetic and Muscular Disorders Research
- Skin and Cellular Biology Research
- Redox biology and oxidative stress
- Endoplasmic Reticulum Stress and Disease
- Polyamine Metabolism and Applications
Jožef Stefan Institute
2016-2020
University of Ljubljana
2019-2020
Institute for Biomedicine
2020
The GGGGCC (G4C2) repeat expansion mutation in C9ORF72 gene is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Transcription formation nuclear RNA foci, which sequester specific RNA-binding proteins one possible pathological mechanisms. Here, we show that (G4C2)n predominantly associates with essential paraspeckle SFPQ, NONO, RBM14, FUS hnRNPH co-localizes known paraspeckle-associated hLinc-p21. As paraspeckles motor neurons has been...
Cold atmospheric plasma treatment of FlpIn SH-SY5Y cells with an inducible expression G3BP1 results in stress granule assembly resembling a cellular oxidative response that has been shown to be eIF2α-signaling dependent and inhibited by ISRIB inhibitor.
Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder, characterized by cytoplasmic inclusions of RNA-binding protein TDP-43. Despite decades research and identification more than 50 genes associated with amyotrophic (ALS), the cause TDP-43 translocation from nucleus its aggregation in cytoplasm still remains unknown. Our study addressed impact selected ALS-associated on behavior wild-type prone vitro cell models. These were developed deleting nuclear localization signal...