Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2018-12

AUTHORS

Delphine Sapaly, Matthieu Dos Santos, Perrine Delers, Olivier Biondi, Gwendoline Quérol, Léo Houdebine, Kevinee Khoobarry, François Girardet, Philippe Burlet, Anne-Sophie Armand, Christophe Chanoine, Jean-François Bureau, Frédéric Charbonnier, Suzie Lefebvre

ABSTRACT

The hereditary neurodegenerative disorder spinal muscular atrophy (SMA) is characterized by the loss of spinal cord motor neurons and skeletal muscle atrophy. SMA is caused by mutations of the survival motor neuron (SMN) gene leading to a decrease in SMN protein levels. The SMN deficiency alters nuclear body formation and whether it can contribute to the disease remains unclear. Here we screen a series of small-molecules on SMA patient fibroblasts and identify flunarizine that accumulates SMN into Cajal bodies, the nuclear bodies important for the spliceosomal small nuclear RNA (snRNA)-ribonucleoprotein biogenesis. Using histochemistry, real-time RT-PCR and behavioural analyses in a mouse model of SMA, we show that along with the accumulation of SMN into Cajal bodies of spinal cord motor neurons, flunarizine treatment modulates the relative abundance of specific spliceosomal snRNAs in a tissue-dependent manner and can improve the synaptic connections and survival of spinal cord motor neurons. The treatment also protects skeletal muscles from cell death and atrophy, raises the neuromuscular junction maturation and prolongs life span by as much as 40 percent (p < 0.001). Our findings provide a functional link between flunarizine and SMA pathology, highlighting the potential benefits of flunarizine in a novel therapeutic perspective against neurodegenerative diseases. More... »

PAGES

2075

References to SciGraph publications

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/s41598-018-20219-1

    DOI

    http://dx.doi.org/10.1038/s41598-018-20219-1

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1100671545

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/29391529


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    411 https://www.grid.ac/institutes/grid.462098.1 schema:alternateName Institut Cochin
    412 schema:name INSERM U1016, Institut Cochin, Université Paris Descartes, Paris, France
    413 Université Paris Descartes, Sorbonne Paris Cité, UFR des Sciences Fondamentales et Biomédicales, INSERM UMR 1124, F-75006, Paris, France
    414 rdf:type schema:Organization
    415 https://www.grid.ac/institutes/grid.465541.7 schema:alternateName Institut Necker Enfants Malades
    416 schema:name INSERM U1151, Institut Necker Enfants Malades (INEM), Université Paris Descartes, Paris, France
    417 Université Paris Descartes, Sorbonne Paris Cité, UFR des Sciences Fondamentales et Biomédicales, INSERM UMR 1124, F-75006, Paris, France
    418 rdf:type schema:Organization
     




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