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Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration

Bernard-Marissal, Nathalie ; Médard, Jean-Jacques ; Azzedine, Hamid ; Chrast, Roman

In: Brain, 2015, vol. 138, no. 4, p. 875-890

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    Titre
    • Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration
    Auteur
    • Bernard-Marissal, Nathalie. 1 Department of Medical Genetics, University of Lausanne, CH-1005 Lausanne, Switzerland
    • Médard, Jean-Jacques. 1 Department of Medical Genetics, University of Lausanne, CH-1005 Lausanne, Switzerland
    • Azzedine, Hamid. 1 Department of Medical Genetics, University of Lausanne, CH-1005 Lausanne, Switzerland
    • Chrast, Roman. 1 Department of Medical Genetics, University of Lausanne, CH-1005 Lausanne, Switzerland
    Type de document
    • Postprint
    Langue
    • Anglais
    Publié dans
    • Brain, 2015, vol. 138, no. 4, p. 875-890. Oxford University Press
    Autre version électronique
    Identifiant OAI-PMH
    • oai:doc.rero.ch:304672
    Summary
    Mutations in SIGMAR1, which encodes the Sigma 1 receptor, cause a familial form of amyotrophic lateral sclerosis, but the underlying molecular mechanisms are unclear. Bernard-Marissal et al. reveal that disruption of Sigma 1 receptor function disturbs endoplasmic reticulum-mitochondria interactions and functions, resulting in degeneration specifically of motor neurons