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OPMD is due to extension of a polyalanine tract in poly(A) binding protein nuclear 1 (PABPN1). Aggregation of the mutant protein in muscle nuclei is a hallmark of the disease. Previous transcriptomic analyses revealed the consistent deregulation of the ubiquitin-proteasome system (UPS) in OPMD animal models and patients, suggesting a role of this deregulation in OPMD pathogenesis. Subsequent studies proposed that UPS contribution to OPMD involved PABPN1 aggregation. Here, we use a<jats:italic>Drosophila<\/jats:italic>model of OPMD to address the functional importance of UPS deregulation in OPMD. Through genome-wide and targeted genetic screens we identify a large number of UPS components that are involved in OPMD. Half dosage of UPS genes reduces OPMD muscle defects suggesting a pathological increase of UPS activity in the disease. Quantification of proteasome activity confirms stronger activity in OPMD muscles, associated with degradation of myofibrillar proteins. Importantly, improvement of muscle structure and function in the presence of UPS mutants does not correlate with the levels of PABPN1 aggregation, but is linked to decreased degradation of muscle proteins. Oral treatment with the proteasome inhibitor MG132 is beneficial to the OPMD<jats:italic>Drosophila<\/jats:italic>model, improving muscle function although PABPN1 aggregation is enhanced. This functional study reveals the importance of increased UPS activity that underlies muscle atrophy in OPMD. It also provides a proof-of-concept that inhibitors of proteasome activity might be an attractive pharmacological approach for OPMD.<\/jats:p>","DOI":"10.1371\/journal.pgen.1010015","type":"journal-article","created":{"date-parts":[[2022,1,13]],"date-time":"2022-01-13T19:30:49Z","timestamp":1642102249000},"page":"e1010015","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":36,"title":["Activation of the ubiquitin-proteasome system contributes to oculopharyngeal muscular dystrophy through muscle atrophy"],"prefix":"10.1371","volume":"18","author":[{"given":"C\u00e9cile","family":"Ribot","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2312-5370","authenticated-orcid":true,"given":"C\u00e9dric","family":"Soler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aymeric","family":"Chartier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4623-9626","authenticated-orcid":true,"given":"Sandy","family":"Al Hayek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rima","family":"Na\u00eft-Sa\u00efdi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2940-9881","authenticated-orcid":true,"given":"Nicolas","family":"Barbezier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Coux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3731-6979","authenticated-orcid":true,"given":"Martine","family":"Simonelig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2022,1,13]]},"reference":[{"issue":"9","key":"pgen.1010015.ref001","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1038\/nrd.2018.109","article-title":"Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing","volume":"17","author":"B Boland","year":"2018","journal-title":"Nat Rev Drug Discov"},{"issue":"2","key":"pgen.1010015.ref002","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/j.bbadis.2006.10.003","article-title":"Oculopharyngeal muscular dystrophy: recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies","volume":"1772","author":"A Abu-Baker","year":"2007","journal-title":"Biochim Biophys Acta"},{"issue":"1","key":"pgen.1010015.ref003","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1007\/s11910-009-0012-y","article-title":"Oculopharyngeal muscular dystrophy: a polyalanine myopathy","volume":"9","author":"B. Brais","year":"2009","journal-title":"Curr Neurol Neurosci Rep"},{"key":"pgen.1010015.ref004","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1038\/ng0298-164","article-title":"Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy","volume":"18","author":"B Brais","year":"1998","journal-title":"Nature Genetics"},{"issue":"4","key":"pgen.1010015.ref005","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1017\/S0317167100018588","article-title":"An 18 alanine repeat in a severe form of oculopharyngeal muscular dystrophy","volume":"41","author":"L Jouan","year":"2014","journal-title":"Can J Neurol Sci"},{"issue":"2","key":"pgen.1010015.ref006","doi-asserted-by":"crossref","first-page":"175","DOI":"10.3233\/JND-140060","article-title":"PABPN1 (GCN)11 as a Dominant Allele in Oculopharyngeal Muscular Dystrophy -Consequences in Clinical Diagnosis and Genetic Counselling.","volume":"2","author":"P Richard","year":"2015","journal-title":"J Neuromuscul Dis"},{"issue":"1","key":"pgen.1010015.ref007","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1007\/BF00692226","article-title":"Nuclear inclusions in oculopharyngeal dystrophy","volume":"49","author":"FM Tome","year":"1980","journal-title":"Acta Neuropathol (Berl)."},{"key":"pgen.1010015.ref008","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1016\/j.devcel.2005.09.002","article-title":"An essential cytoplasmic function for the nuclear poly(A) binding protein, PABP2, in poly(A) tail length control and early development in Drosophila.","volume":"9","author":"B Benoit","year":"2005","journal-title":"Dev Cell"},{"key":"pgen.1010015.ref009","doi-asserted-by":"crossref","first-page":"3771","DOI":"10.1093\/nar\/27.19.3771","article-title":"The Drosophila poly(A)-binding protein II is ubiquitous throughout Drosophila development and has the same function in mRNA polyadenylation as its bovine homolog in vitro.","volume":"27","author":"B Benoit","year":"1999","journal-title":"Nucleic Acids Res"},{"issue":"14","key":"pgen.1010015.ref010","doi-asserted-by":"crossref","first-page":"3705","DOI":"10.1093\/emboj\/cdg347","article-title":"Stimulation of poly(A) polymerase through a direct interaction with the nuclear poly(A) binding protein allosterically regulated by RNA.","volume":"22","author":"Y Kerwitz","year":"2003","journal-title":"The EMBO journal"},{"issue":"34","key":"pgen.1010015.ref011","doi-asserted-by":"crossref","first-page":"22803","DOI":"10.1074\/jbc.M109.018226","article-title":"Poly(A) tail length is controlled by the nuclear poly(A)-binding protein regulating the interaction between poly(A) polymerase and the cleavage and polyadenylation specificity factor.","volume":"284","author":"U Kuhn","year":"2009","journal-title":"The Journal of biological chemistry"},{"key":"pgen.1010015.ref012","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/0092-8674(91)90119-J","article-title":"A novel poly(A)-Binding protein acts as a specificity factor in the second phase of messenger RNA polyadenylation","volume":"66","author":"E. 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pathology.","volume":"26","author":"KE Vest","year":"2017","journal-title":"Hum Mol Genet"},{"issue":"15","key":"pgen.1010015.ref025","doi-asserted-by":"crossref","first-page":"2321","DOI":"10.1093\/oxfordjournals.hmg.a018924","article-title":"Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein II aggregates which sequester poly(A) RNA.","volume":"9","author":"A Calado","year":"2000","journal-title":"Hum Mol Genet"},{"issue":"3","key":"pgen.1010015.ref026","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/j.nbd.2004.10.019","article-title":"PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions","volume":"18","author":"LP Corbeil-Girard","year":"2005","journal-title":"Neurobiol Dis"},{"issue":"3","key":"pgen.1010015.ref027","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1017\/S0317167100002675","article-title":"HnRNP A1 and A\/B interaction with PABPN1 in oculopharyngeal muscular dystrophy","volume":"30","author":"X Fan","year":"2003","journal-title":"Can J Neurol Sci"},{"issue":"22","key":"pgen.1010015.ref028","doi-asserted-by":"crossref","first-page":"10929","DOI":"10.1093\/nar\/gkw703","article-title":"Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.","volume":"44","author":"P Klein","year":"2016","journal-title":"Nucleic Acids Res"},{"issue":"6","key":"pgen.1010015.ref029","first-page":"412","article-title":"A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging.","volume":"5","author":"SY Anvar","year":"2013","journal-title":"Aging (Albany NY).PubMed Central PMCID"},{"issue":"1","key":"pgen.1010015.ref030","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/emmm.201000109","article-title":"Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 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a transgenic mouse model of oculopharyngeal muscular dystrophy","volume":"15","author":"JE Davies","year":"2006","journal-title":"Hum Mol Genet"},{"issue":"6","key":"pgen.1010015.ref034","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1038\/nm1242","article-title":"Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice","volume":"11","author":"JE Davies","year":"2005","journal-title":"Nat Med"},{"issue":"4","key":"pgen.1010015.ref035","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1016\/j.ajpath.2013.12.011","article-title":"A Novel Feed-Forward Loop between ARIH2 E3-Ligase and PABPN1 Regulates Aging-Associated Muscle Degeneration","volume":"184","author":"V Raz","year":"2014","journal-title":"Am J Pathol"},{"issue":"4","key":"pgen.1010015.ref036","doi-asserted-by":"crossref","first-page":"1988","DOI":"10.1016\/j.ajpath.2011.06.044","article-title":"Modeling oculopharyngeal muscular dystrophy in myotube 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Riaz","year":"2016","journal-title":"PLoS Genet."},{"issue":"8","key":"pgen.1010015.ref067","doi-asserted-by":"crossref","first-page":"325","DOI":"10.2183\/pjab.94.021","article-title":"Transcriptional regulation of the 26S proteasome by Nrf1","volume":"94","author":"S Koizumi","year":"2018","journal-title":"Proc Jpn Acad Ser B Phys Biol Sci"},{"issue":"23","key":"pgen.1010015.ref068","doi-asserted-by":"crossref","first-page":"8786","DOI":"10.1128\/MCB.23.23.8786-8794.2003","article-title":"Antioxidants enhance mammalian proteasome expression through the Keap1-Nrf2 signaling pathway","volume":"23","author":"MK Kwak","year":"2003","journal-title":"Mol Cell Biol"},{"issue":"4","key":"pgen.1010015.ref069","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1128\/MCB.00799-10","article-title":"Basic leucine zipper protein Cnc-C is a substrate and transcriptional regulator of the Drosophila 26S proteasome","volume":"31","author":"KB Grimberg","year":"2011","journal-title":"Mol Cell 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Arrasate","year":"2004","journal-title":"Nature"},{"issue":"5","key":"pgen.1010015.ref073","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1002\/jcsm.12438","article-title":"Inhibition of myostatin improves muscle atrophy in oculopharyngeal muscular dystrophy (OPMD).","volume":"10","author":"P Harish","year":"2019","journal-title":"J Cachexia Sarcopenia Muscle"},{"issue":"7","key":"pgen.1010015.ref074","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1038\/nrclinonc.2016.206","article-title":"Proteasome inhibitors in cancer therapy","volume":"14","author":"EE Manasanch","year":"2017","journal-title":"Nat Rev Clin Oncol"},{"key":"pgen.1010015.ref075","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/978-1-4939-6771-1_5","article-title":"Characterization of Drosophila Muscle Stem Cell-Like Adult Muscle Precursors.","volume":"1556","author":"G Lavergne","year":"2017","journal-title":"Methods Mol 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Res"},{"issue":"13","key":"pgen.1010015.ref079","doi-asserted-by":"crossref","first-page":"2309","DOI":"10.1128\/MCB.01346-14","article-title":"General and MicroRNA-Mediated mRNA Degradation Occurs on Ribosome Complexes in Drosophila Cells.","volume":"35","author":"S Antic","year":"2015","journal-title":"Mol Cell Biol"},{"issue":"4","key":"pgen.1010015.ref080","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1128\/MCB.01227-08","article-title":"Genetic evidence linking age-dependent attenuation of the 26S proteasome with the aging process","volume":"29","author":"A Tonoki","year":"2009","journal-title":"Mol Cell Biol"},{"issue":"9","key":"pgen.1010015.ref081","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1016\/j.bbamcr.2007.05.007","article-title":"Adaptive modification and flexibility of the proteasome system in response to proteasome inhibition.","volume":"1773","author":"C Naujokat","year":"2007","journal-title":"Biochim Biophys 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