In Vitro and in Cellulo Evidences for Association of the Survival of Motor Neuron Complex with the Fragile X Mental Retardation Protein
| Autor: | Séverine Massenet, Hervé Moine, Florence Rage, Christiane Branlant, Nathalie Piazzon, Florence Schlotter |
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| Přispěvatelé: | Institut de génétique et biologie moléculaire et cellulaire (IGBMC), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Louis Pasteur - Strasbourg I, Maturation des ARN et enzymologie moléculaire (MAEM), Cancéropôle du Grand Est-Université Henri Poincaré - Nancy 1 (UHP)-IFR111-Centre National de la Recherche Scientifique (CNRS), Institut de Génétique Moléculaire de Montpellier (IGMM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Université Louis Pasteur - Strasbourg I-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), MASSENET, Séverine |
| Rok vydání: | 2008 |
| Předmět: |
[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology
[SDV]Life Sciences [q-bio] animal diseases Biochemistry Fragile X Mental Retardation Protein Mice MESH: Protein Structure Tertiary 0302 clinical medicine SMN complex MESH: Muscular Atrophy Spinal Translational regulation MESH: Animals MESH: Nerve Tissue Proteins Cyclic AMP Response Element-Binding Protein Motor Neurons 0303 health sciences RNA-Binding Proteins SMN Complex Proteins Exons Cell biology medicine.anatomical_structure MESH: Fragile X Syndrome MESH: Motor Neurons MESH: Cyclic AMP Response Element-Binding Protein congenital hereditary and neonatal diseases and abnormalities MESH: Cell Line Tumor Immunoprecipitation Recombinant Fusion Proteins Hypothalamus Nerve Tissue Proteins Biology MESH: Fragile X Mental Retardation Protein Muscular Atrophy Spinal 03 medical and health sciences Cell Line Tumor MESH: Recombinant Fusion Proteins medicine Animals Humans MRNA transport MESH: Mice Molecular Biology 030304 developmental biology MESH: Humans [SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry Molecular Biology/Molecular biology Survival of motor neuron Cell Biology Spinal muscular atrophy Motor neuron medicine.disease Survival of Motor Neuron 1 Protein MESH: Hypothalamus Molecular biology Protein Structure Tertiary nervous system diseases MESH: RNA-Binding Proteins nervous system Fragile X Syndrome MESH: Exons 030217 neurology & neurosurgery |
| Zdroj: | Journal of Biological Chemistry Journal of Biological Chemistry, American Society for Biochemistry and Molecular Biology, 2008, 283 (9), pp.5598-610. ⟨10.1074/jbc.M707304200⟩ Journal of Biological Chemistry, American Society for Biochemistry and Molecular Biology, 2008, 283 (9), pp.5598-5610. ⟨10.1074/jbc.M707304200⟩ Journal of Biological Chemistry, 2008, 283 (9), pp.5598-5610. ⟨10.1074/jbc.M707304200⟩ |
| ISSN: | 0021-9258 1083-351X |
| Popis: | International audience; Spinal muscular atrophy (SMA) is caused by reduced levels of the survival of motor neuron (SMN) protein. Although the SMN complex is essential for assembly of spliceosomal U small nuclear RNPs, it is still not understood why reduced levels of the SMN protein specifically cause motor neuron degeneration. SMN was recently proposed to have specific functions in mRNA transport and translation regulation in neuronal processes. The defective protein in Fragile X mental retardation syndrome (FMRP) also plays a role in transport of mRNPs and in their translation. Therefore, we examined possible relationships of SMN with FMRP. We observed granules containing both transiently expressed red fluorescent protein(RFP)-tagged SMN and green fluorescent protein(GFP)-tagged FMRP in cell bodies and processes of rat primary neurons of hypothalamus in culture. By immunoprecipitation experiments, we detected an association of FMRP with the SMN complex in human neuroblastoma SH-SY5Y cells and in murine motor neuron MN-1 cells. Then, by in vitro experiments, we demonstrated that the SMN protein is essential for this association. We showed that the COOH-terminal region of FMRP, as well as the conserved YG box and the region encoded by exon 7 of SMN, are required for the interaction. Our findings suggest a link between the SMN complex and FMRP in neuronal cells. |
| Databáze: | OpenAIRE |
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