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• W2133736734 endingPage "1795" @default.
• W2133736734 startingPage "1785" @default.
• W2133736734 abstract "The survival of motor neuron (SMN) protein is mutated in patients with spinal muscular atrophy (SMA). SMN is part of a multiprotein complex required for biogenesis of the Sm class of small nuclear ribonucleoproteins (snRNPs). Following assembly of the Sm core domain, snRNPs are transported to the nucleus via importin β. Sm snRNPs contain a nuclear localization signal (NLS) consisting of a 2,2,7-trimethylguanosine (TMG) cap and the Sm core. Snurportin1 (SPN) is the adaptor protein that recognizes both the TMG cap and importin β. Here, we report that a mutant SPN construct lacking the importin β binding domain (IBB), but containing an intact TMG cap-binding domain, localizes primarily to the nucleus, whereas full-length SPN localizes to the cytoplasm. The nuclear localization of the mutant SPN was not a result of passive diffusion through the nuclear pores. Importantly, we found that SPN interacts with SMN, Gemin3, Sm snRNPs and importin β. In the presence of ribonucleases, the interactions with SMN and Sm proteins were abolished, indicating that snRNAs mediate this interplay. Cell fractionation studies showed that SPN binds preferentially to cytoplasmic SMN complexes. Notably, we found that SMN directly interacts with importin β in a GST-pulldown assay, suggesting that the SMN complex might represent the Sm core NLS receptor predicted by previous studies. Therefore, we conclude that, following Sm protein assembly, the SMN complex persists until the final stages of cytoplasmic snRNP maturation and may provide somatic cell RNPs with an alternative NLS." @default.
• W2133736734 created "2016-06-24" @default.
• W2133736734 creator A5021502226 @default.
• W2133736734 creator A5038388378 @default.
• W2133736734 creator A5065194210 @default.
• W2133736734 creator A5081559424 @default.
• W2133736734 creator A5083677717 @default.
• W2133736734 date "2002-07-15" @default.
• W2133736734 modified "2023-10-17" @default.
• W2133736734 title "SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta" @default.
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• W2133736734 doi "https://doi.org/10.1093/hmg/11.15.1785" @default.
• W2133736734 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/1630493" @default.
• W2133736734 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/12095920" @default.
• W2133736734 hasPublicationYear "2002" @default.
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• W2133736734 hasAuthorship W2133736734A5065194210 @default.
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