期刊
RESPIRATORY PHYSIOLOGY & NEUROBIOLOGY
卷 168, 期 1-2, 页码 109-118出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.resp.2009.05.013
关键词
Hypoxia; Hypercapnia; Mecp2; Rett syndrome; Tidal volume
资金
- CNRS
- Universite de la Mediterranee
- Universite Paul Cezanne
- French Ministry for Research [054]
- Rett Syndrome Research Foundation
- International Rett Syndrome Foundation
Rett syndrome (RTT) is a rare neurodevelopmental disease caused by mutations in the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2) and accompanied by complex symptoms, including erratic breathing and life-threatening apnoeas. In Mecp2-deficient male mice (Mecp2(-/y)), breathing is normal at birth but becomes altered after postnatal day 30 (P30), with erratic rhythm and apnoeas aggravating until death at around P60. Using plethysmography, we analyzed breathing of unrestrained wild type mice and Mecp2(-/y) at P15, P25 and P30 under air and under short-lasting exposure to moderate hypoxia or hypercapnia. In Mecp2(-/y) with normal resting ventilation, we report exacerbated respiratory responses to hypoxia at P30 and transient apnoeas with erratic rhythm after hypoxia and hypercapnia at P30, P25 and occasionally P15. Then environmental factors may induce breathing defects well before than expected in Mecp2(-/y) and possibly in RTT patients. We therefore suggest avoiding exposure of young RTT patients to environmental situations where they may encounter moderate hypoxia or hypercapnia. (C) 2009 Elsevier B.V. All rights reserved.
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