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Early abnormalities of post-sigh breathing in a mouse model of Rett syndrome.
- Source :
-
Respiratory physiology & neurobiology [Respir Physiol Neurobiol] 2010 Feb 28; Vol. 170 (2), pp. 173-82. Date of Electronic Publication: 2009 Dec 28. - Publication Year :
- 2010
-
Abstract
- 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.<br /> (Copyright 2010 Elsevier B.V. All rights reserved.)
- Subjects :
- Age Factors
Animals
Animals, Newborn
Apnea genetics
Apnea physiopathology
Disease Models, Animal
Hypercalcemia genetics
Hypoxia genetics
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Plethysmography
Respiration genetics
Respiratory Mechanics genetics
Respiratory System Abnormalities genetics
Rett Syndrome genetics
Tidal Volume genetics
Hypercalcemia complications
Hypoxia complications
Methyl-CpG-Binding Protein 2 deficiency
Respiratory System Abnormalities physiopathology
Rett Syndrome physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1878-1519
- Volume :
- 170
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Respiratory physiology & neurobiology
- Publication Type :
- Academic Journal
- Accession number :
- 20040383
- Full Text :
- https://doi.org/10.1016/j.resp.2009.12.009