Early abnormalities of post-sigh breathing in a mouse model of Rett syndrome
| Autor: | Gérard Hilaire, Sebastien Zanella, Mathias Dutschmann, Nicolas Voituron, Clément Menuet, Anne-Marie Lajard |
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| Přispěvatelé: | Centre de recherche en neurobiologie - neurophysiologie de Marseille (CRN2M), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS) |
| Rok vydání: | 2010 |
| Předmět: |
Male
Apnea Methyl-CpG-Binding Protein 2 Physiology MESH: Anoxia MESH: Mice Knockout MESH: Tidal Volume MESH: Methyl-CpG-Binding Protein 2 MESH: Animals Newborn Mice 0302 clinical medicine MESH: Animals Hypoxia Tidal volume MESH: Rett Syndrome Mice Knockout 0303 health sciences MESH: Apnea Eupnea Respiration General Neuroscience Age Factors Plethysmography medicine.anatomical_structure Cardiology Breathing MESH: Respiratory Mechanics Respiratory System Abnormalities medicine.symptom Hypercapnia Pulmonary and Respiratory Medicine congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Rett syndrome Biology MESH: Hypercalcemia MECP2 03 medical and health sciences MESH: Mice Inbred C57BL Internal medicine MESH: Plethysmography mental disorders Rett Syndrome Tidal Volume medicine Animals [SDV.BBM.BC]Life Sciences [q-bio]/Biochemistry Molecular Biology/Biochemistry [q-bio.BM] MESH: Mice 030304 developmental biology MESH: Age Factors MESH: Respiration Lung Hypoxia (medical) medicine.disease MESH: Male nervous system diseases Mice Inbred C57BL Disease Models Animal Endocrinology Animals Newborn Hypercalcemia Respiratory Mechanics MESH: Disease Models Animal MESH: Respiratory System Abnormalities 030217 neurology & neurosurgery |
| Zdroj: | Respiratory Physiology & Neurobiology Respiratory Physiology & Neurobiology, 2010, 170 (2), pp.173-82. ⟨10.1016/j.resp.2009.12.009⟩ Respiratory Physiology and Neurobiology Respiratory Physiology and Neurobiology, Elsevier, 2010, 170 (2), pp.173-82. ⟨10.1016/j.resp.2009.12.009⟩ |
| ISSN: | 1569-9048 1878-1519 |
| DOI: | 10.1016/j.resp.2009.12.009 |
| Popis: | International audience; Rett syndrome is a neurodevelopmental disease accompanied by complex, disabling symptoms, including breathing symptoms. Because Rett syndrome is caused by mutations in the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2), Mecp2-deficient mice have been generated as experimental model. Males of Mecp2-deficient mice (Mecp2(-/y)) breathe normally at birth but show abnormal respiratory responses to hypoxia and hypercapnia from postnatal day 25 (P25). After P30, Mecp2(-/y) mice develop breathing symptoms reminiscent of Rett syndrome, aggravating until premature death at around P60. Using plethysmography, we analyzed the sighs and the post-sigh breathing pattern of unrestrained wild type male mice (WT) and Mecp2(-/y) mice from P15 to P60. Sighs are spontaneous large inspirations known to prevent lung atelectasis and to improve alveolar oxygenation. However, Mecp2(-/y) mice show early abnormalities of post-sigh breathing, with long-lasting post-sigh apnoeas, reduced tidal volume when eupnoea resumes and lack of post-sigh bradypnoea which develop from P15, aggravate with age and possibly contribute to breathing symptoms to come. |
| Databáze: | OpenAIRE |
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