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Alliance"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:p>Although recent advances in gene therapy provide hope for spinal muscular atrophy (SMA) patients, the pathology remains the leading genetic cause of infant mortality. SMA is a monogenic pathology that originates from the loss of the<jats:italic>SMN1<\/jats:italic>gene in most cases or mutations in rare cases. Interestingly, several<jats:italic>SMN1<\/jats:italic>mutations occur within the TUDOR methylarginine reader domain of SMN. We hypothesized that in<jats:italic>SMN1<\/jats:italic>mutant cases, SMA may emerge from aberrant protein-protein interactions between SMN and key neuronal factors. Using a BioID proteomic approach, we have identified and validated a number of SMN-interacting proteins, including fragile X mental retardation protein (FMRP) family members (FMR<jats:sub>FM<\/jats:sub>). Importantly, SMA-linked SMN<jats:sub>TUDOR<\/jats:sub>mutant forms (SMN<jats:sub>ST<\/jats:sub>) failed to interact with FMR<jats:sub>FM<\/jats:sub>. In agreement with the recent work, we define biochemically that SMN forms droplets in vitro and these droplets are stabilized by RNA, suggesting that SMN could be involved in the formation of membraneless organelles, such as Cajal nuclear bodies. Finally, we found that SMN and FMRP co-fractionate with polysomes, in an RNA-dependent manner, suggesting a potential role in localized translation in motor neurons.<\/jats:p>","DOI":"10.26508\/lsa.202201429","type":"journal-article","created":{"date-parts":[[2022,11,14]],"date-time":"2022-11-14T15:10:44Z","timestamp":1668438644000},"page":"e202201429","update-policy":"https:\/\/doi.org\/10.26508\/lsa.crossmarkpolicy","source":"Crossref","is-referenced-by-count":17,"title":["SMA-linked SMN mutants prevent phase separation properties and SMN interactions with FMRP family members"],"prefix":"10.26508","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1539-0828","authenticated-orcid":true,"given":"Olivier","family":"Binda","sequence":"first","affiliation":[{"name":"Universit\u00e9 Claude Bernard Lyon 1, CNRS UMR 5261, INSERM U1315, LabEx DEV2CAN, Institut NeuroMyoG\u00e8ne-Pathophysiology and Genetics of Neuron and Muscle, Team Chromatin Dynamics, Nuclear Domains, Virus, 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