Ontology highlight
ABSTRACT:
INSTRUMENT(S): impact II
ORGANISM(S): Homo Sapiens (human)
TISSUE(S): Mesenchymal Stem Cell, Stem Cell
SUBMITTER: Kamil Mikulasek
LAB HEAD: Zbynek Zdrahal
PROVIDER: PXD012709 | Pride | 2020-02-18
REPOSITORIES: Pride
Laidou Stamatia S Alanis-Lobato Gregorio G Pribyl Jan J Raskó Tamás T Tichy Boris B Mikulasek Kamil K Tsagiopoulou Maria M Oppelt Jan J Kastrinaki Georgia G Lefaki Maria M Singh Manvendra M Zink Annika A Chondrogianni Niki N Psomopoulos Fotis F Prigione Alessandro A Ivics Zoltán Z Pospisilova Sarka S Skladal Petr P Izsvák Zsuzsanna Z Andrade-Navarro Miguel A MA Petrakis Spyros S
Redox biology 20200211
Spinocerebellar ataxia type-1 (SCA1) is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are somehow linked to neuronal death. However, owing to lack of a suitable cellular model, the downstream consequences of IIB formation are yet to be resolved. Here, we describe a nuclear protein aggregation model of pathogenic human ataxin-1 and characterize IIB e ...[more]