CLIPR-59: a protein essential for neuromuscular junction stability during mouse late embryonic development
| Autor: | Véronique Bernard, Stéphanie Backer, Evelyne Bloch-Gallego, Nathalie Chaverot, Ariane Dimitrov, Jordi Molgó, Franck Perez, Nicolas Offner, Aurélie Couesnon |
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| Přispěvatelé: | Institut Cochin (UMR_S567 / UMR 8104), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie des Maladies du Système Nerveux Central, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Compartimentation et dynamique cellulaires (CDC), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut Curie [Paris]-Centre National de la Recherche Scientifique (CNRS), Neurobiologie et Développement (N&eD), Centre National de la Recherche Scientifique (CNRS), Institut de Neurobiologie Alfred Fessard (INAF), Centre National de la Recherche Scientifique (CNRS)-Institut Curie [Paris]-Université Pierre et Marie Curie - Paris 6 (UPMC), Neurosciences Paris Seine (NPS), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Biologie Paris Seine (IBPS), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), CNRS, Laboratoire de Neurobiologie et Développement, Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5), Centre National de la Recherche Scientifique (CNRS)-Institut Curie-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Paris-Sud - Paris 11 (UP11)-Centre National de la Recherche Scientifique (CNRS), Institut Cochin ( UMR_S567 / UMR 8104 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Compartimentation et dynamique cellulaires ( CDC ), Centre National de la Recherche Scientifique ( CNRS ) -INSTITUT CURIE-Université Pierre et Marie Curie - Paris 6 ( UPMC ), Neurobiologie et Développement ( N&eD ), Université Paris-Sud - Paris 11 ( UP11 ) -Centre National de la Recherche Scientifique ( CNRS ), Institut de Neurobiologie Alfred Fessard ( INAF ), Centre National de la Recherche Scientifique ( CNRS ) |
| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
Mutant
MESH: Spinal Cord Mice MESH : Embryonic Development 0302 clinical medicine MESH: Pregnancy Pregnancy MESH: Gestational Age MESH : Embryo Mammalian Homeostasis MESH: Embryonic Development MESH : Female MESH: Animals Axon Respiratory system ComputingMilieux_MISCELLANEOUS Cells Cultured 0303 health sciences Brain Anatomy MESH : Mice Transgenic Cell biology medicine.anatomical_structure Spinal Cord MESH: Homeostasis MESH : Homeostasis Knockout mouse Female [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] Microtubule-Associated Proteins MESH: Cells Cultured MESH: Mice Transgenic Neuromuscular Junction Embryonic Development Gestational Age Mice Transgenic MESH : Mice Inbred C57BL Biology Contractility 03 medical and health sciences MESH: Brain MESH: Mice Inbred C57BL MESH : Cells Cultured MESH : Mice medicine Animals Molecular Biology MESH: Mice 030304 developmental biology Embryogenesis MESH: Embryo Mammalian MESH : Spinal Cord Embryo Mammalian Embryonic stem cell Mice Inbred C57BL MESH : Pregnancy MESH: Microtubule-Associated Proteins MESH : Brain nervous system MESH : Microtubule-Associated Proteins [ SDV.NEU ] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] MESH : Neuromuscular Junction Axon guidance MESH : Animals MESH: Neuromuscular Junction MESH: Female 030217 neurology & neurosurgery MESH : Gestational Age Developmental Biology |
| Zdroj: | Development (Cambridge, England) Development (Cambridge, England), 2013, 140 (7), pp.1583-93. ⟨10.1242/dev.087106⟩ Development (Cambridge, England), Company of Biologists, 2013, 140 (7), pp.1583-93. ⟨10.1242/dev.087106⟩ Development (Cambridge, England), Company of Biologists, 2013, 140 (7), pp.1583-1593. ⟨10.1242/dev.087106⟩ Development (Cambridge, England), Company of Biologists, 2013, 140 (7), pp.1583-93. 〈10.1242/dev.087106〉 |
| ISSN: | 0950-1991 1477-9129 |
| Popis: | International audience; CLIPR-59 is a new member of the cytoplasmic linker proteins (CLIP) family mainly localized to the trans-Golgi network. We show here that Clipr-59 expression in mice is restricted to specific pools of neurons, in particular motoneurons (MNs), and progressively increases from embryonic day 12.5 (E12.5) until the first postnatal days. We generated a Clipr-59 knockout mouse model that presents perinatal lethality due to respiratory defects. Physiological experiments revealed that this altered innervation prevents the normal nerve-elicited contraction of the mutant diaphragm that is reduced both in amplitude and fatigue-resistance at E18.5, despite unaffected functional muscular contractility. Innervation of the mutant diaphragm is not altered until E15.5, but is then partially lost in the most distal parts of the muscle. Ultrastructural observations of neuromuscular junctions (NMJs) in the distal region of the diaphragm reveal a normal organization, but a lower density of nerve terminals capped by terminal Schwann cells in E18.5 mutant when compared with control embryos. Similar defects in NMJ stability, with a hierarchy of severity along the caudo-rostral axis, are also observed in other muscles innervated by facial and spinal MNs in Clipr-59 mutant mice. Clipr-59 deficiency therefore affects axon maintenance but not axon guidance toward muscle targets. Thus, CLIPR-59 is involved in the stabilization of specific motor axons at the NMJ during mouse late embryogenesis and its role is crucial for mouse perinatal development. |
| Databáze: | OpenAIRE |
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