{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["de Chiara C"],"funding":["Medical Research Council"],"pagination":["211-8"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC3988977"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["37(4)"],"pubmed_abstract":["Understanding how proteins protect themselves from aberrant aggregation is of primary interest for understanding basic biology, protein biochemistry, and human disease. We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegenerative spinocerebellar ataxia type 1 (SCA1). This disease is part of the increasing family of pathologies caused by protein aggregation and misfolding. We discuss the importance of protein-protein interactions not only in the nonpathological function of Atx1 but also in protecting the protein from aggregation and misfolding. The lessons learned from Atx1 may lead to a more general understanding of the cell's protective strategies against aggregation. The obtained knowledge may suggest a new perspective for designing specific therapeutic strategies for the cure of misfolding diseases."],"journal":["Trends in neurosciences"],"pubmed_title":["Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation."],"pmcid":["PMC3988977"],"funding_grant_id":["MC_PC_13054","MC_U117584256","U117584256"],"pubmed_authors":["de Chiara C","Pastore A"],"additional_accession":[]},"is_claimable":false,"name":"Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.","description":"Understanding how proteins protect themselves from aberrant aggregation is of primary interest for understanding basic biology, protein biochemistry, and human disease. We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegenerative spinocerebellar ataxia type 1 (SCA1). This disease is part of the increasing family of pathologies caused by protein aggregation and misfolding. We discuss the importance of protein-protein interactions not only in the nonpathological function of Atx1 but also in protecting the protein from aggregation and misfolding. The lessons learned from Atx1 may lead to a more general understanding of the cell's protective strategies against aggregation. The obtained knowledge may suggest a new perspective for designing specific therapeutic strategies for the cure of misfolding diseases.","dates":{"release":"2014-01-01T00:00:00Z","publication":"2014 Apr","modification":"2024-10-14T23:22:25.596Z","creation":"2019-03-27T01:25:10Z"},"accession":"S-EPMC3988977","cross_references":{"pubmed":["24636457"],"doi":["10.1016/j.tins.2014.02.003"]}}