title: In vivo time-lapse imaging of mitochondria in healthy and diseased peripheral myelin sheath.
abstract: The myelin sheath that covers a large amount of neurons is critical for their homeostasis, and myelinating glia mitochondria have recently been shown to be essential for neuron survival. However morphological and physiological properties of these organelles remain elusive. Here we report a method to analyze mitochondrial dynamics and morphology in myelinating Schwann cells of living mice using viral transduction and time-lapse multiphoton microscopy. We describe the distribution, shape, size and dynamics of mitochondria in live cells. We also report mitochondrial alterations in Opa1(delTTAG) mutant mice cells at presymptomatic stages, suggesting that mitochondrial defects in myelin contribute to OPA1 related neuropathy and represent a biomarker for the disease.
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France.
authors.forename: Sergio
authors.initials: S
authors.lastname: Gonzalez
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France.
authors.forename: Ruani
authors.initials: R
authors.lastname: Fernando
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France.
authors.forename: Jade
authors.initials: J
authors.lastname: Berthelot
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Perrin-Tricaud
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Sarzi
authors.affiliation: Karolinska Institutet, Department of Clinical Neuroscience, Department of Neuroscience, Stockhom 171 77, Sweden.
authors.forename: Roman
authors.initials: R
authors.lastname: Chrast
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France; Mitochondrial Medicine Research Centre, Pôle de Recherche et d'Enseignement en Médecine Mitochondriale, Université d'Angers, Angers 49933, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Lenaers
authors.affiliation: INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France. Electronic address: nicolas.tricaud@inserm.fr.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Tricaud
mesh_terms: D000818:Animals; D020558:GTP Phosphohydrolases / Q000172:deficiency; D051379:Mice; D036641:Microscopy, Fluorescence, Multiphoton; D008928:Mitochondria / Q000378:metabolism* / Q000648:ultrastructure*; D009186:Myelin Sheath / Q000502:physiology* / Q000648:ultrastructure*; D012583:Schwann Cells / Q000502:physiology* / Q000648:ultrastructure*; D059008:Time-Lapse Imaging
chemical_list: D020558:GTP Phosphohydrolases; C498809:Opa1 protein, mouse
keywords: In vivo imaging; Multiphoton microscopy; Myelin; Opa1; Schwann cell mitochondria