1. Nucleolar reorganization after cellular stress is orchestrated by SMN shuttling between nuclear compartments.
- Author
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Musawi S, Donnio LM, Zhao Z, Magnani C, Rassinoux P, Binda O, Huang J, Jacquier A, Coudert L, Lomonte P, Martinat C, Schaeffer L, Mottet D, Côté J, Mari PO, and Giglia-Mari G
- Subjects
- Humans, Nerve Tissue Proteins metabolism, Cell Nucleolus metabolism, Motor Neurons metabolism, SMN Complex Proteins metabolism, Coiled Bodies metabolism, Protein-Arginine N-Methyltransferases metabolism, Repressor Proteins metabolism, RNA-Binding Proteins metabolism, Muscular Atrophy, Spinal genetics, Muscular Atrophy, Spinal metabolism
- Abstract
Spinal muscular atrophy is an autosomal recessive neuromuscular disease caused by mutations in the multifunctional protein Survival of Motor Neuron, or SMN. Within the nucleus, SMN localizes to Cajal bodies, which are associated with nucleoli, nuclear organelles dedicated to the first steps of ribosome biogenesis. The highly organized structure of the nucleolus can be dynamically altered by genotoxic agents. RNAP1, Fibrillarin, and nucleolar DNA are exported to the periphery of the nucleolus after genotoxic stress and, once DNA repair is fully completed, the organization of the nucleolus is restored. We find that SMN is required for the restoration of the nucleolar structure after genotoxic stress. During DNA repair, SMN shuttles from the Cajal bodies to the nucleolus. This shuttling is important for nucleolar homeostasis and relies on the presence of Coilin and the activity of PRMT1., (© 2023. The Author(s).)
- Published
- 2023
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