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We previously demonstrated that FMRP binds mRNAs targeted for nonsense-mediated mRNA decay (NMD) and that FMRP loss results in hyperactivated NMD and inhibition of neuronal differentiation in human stem cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We show here that NMD is hyperactivated during the development of the cerebral cortex, hippocampus, and cerebellum in the<jats:italic>Fmr1<\/jats:italic>-knockout (KO) mouse during embryonic and early postnatal periods. Our findings demonstrate that NMD regulates many neuronal mRNAs that are important for mouse brain development.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We reveal the abnormal regulation of these mRNAs in the<jats:italic>Fmr1<\/jats:italic>-KO mouse, a model of FXS, and highlight the importance of early intervention.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s13059-021-02530-9","type":"journal-article","created":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T14:04:23Z","timestamp":1637071463000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["NMD abnormalities during brain development in the Fmr1-knockout mouse model of fragile X syndrome"],"prefix":"10.1186","volume":"22","author":[{"given":"Tatsuaki","family":"Kurosaki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitomi","family":"Sakano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Pr\u00f6schel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason","family":"Wheeler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Hewko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2789-2075","authenticated-orcid":false,"given":"Lynne E.","family":"Maquat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,11,16]]},"reference":[{"issue":"4","key":"2530_CR1","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1007\/s12551-020-00730-4","volume":"12","author":"C Malecki","year":"2020","unstructured":"Malecki C, Hambly BD, Jeremy RW, Robertson EN. 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