{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["12(2)"],"submitter":["Chen ZS"],"pubmed_abstract":["Polyglutamine (polyQ) diseases comprise Huntington's disease and several subtypes of spinocerebellar ataxia, including spinocerebellar ataxia type 3 (SCA3). The genomic expansion of coding CAG trinucleotide sequence in disease genes leads to the production and accumulation of misfolded polyQ domain-containing disease proteins, which cause cellular dysfunction and neuronal death. As one of the principal cellular protein clearance pathways, the activity of the ubiquitin-proteasome system (UPS) is tightly regulated to ensure efficient clearance of damaged and toxic proteins. Emerging evidence demonstrates that UPS plays a crucial role in the pathogenesis of polyQ diseases. Ubiquitin (Ub) E3 ligases catalyze the transfer of a Ub tag to label proteins destined for proteasomal clearance. In this"],"journal":["Cell death & disease"],"pagination":["136"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7862454"],"repository":["biostudies-literature"],"pubmed_title":["A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3."],"pmcid":["PMC7862454"],"pubmed_authors":["Chen ZS","Talbot K","Huang X","Chan HYE"],"additional_accession":[]},"is_claimable":false,"name":"A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3.","description":"Polyglutamine (polyQ) diseases comprise Huntington's disease and several subtypes of spinocerebellar ataxia, including spinocerebellar ataxia type 3 (SCA3). The genomic expansion of coding CAG trinucleotide sequence in disease genes leads to the production and accumulation of misfolded polyQ domain-containing disease proteins, which cause cellular dysfunction and neuronal death. As one of the principal cellular protein clearance pathways, the activity of the ubiquitin-proteasome system (UPS) is tightly regulated to ensure efficient clearance of damaged and toxic proteins. Emerging evidence demonstrates that UPS plays a crucial role in the pathogenesis of polyQ diseases. Ubiquitin (Ub) E3 ligases catalyze the transfer of a Ub tag to label proteins destined for proteasomal clearance. In this","dates":{"release":"2021-01-01T00:00:00Z","publication":"2021 Feb","modification":"2025-04-04T10:06:04.778Z","creation":"2021-02-21T10:30:45Z"},"accession":"S-EPMC7862454","cross_references":{"pubmed":["33542212"],"doi":["10.1038/s41419-021-03444-x"]}}