Physiological definition of upper airway obstructions in mouse model for Rett syndrome.
Summary of "Physiological definition of upper airway obstructions in mouse model for Rett syndrome."
Rett syndrome is a neuro-developmental disease accompanied by breathing symptoms including breath-hold events, and is caused by mutation of the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2). Males of Mecp2-deficient mice (Mecp2(-/y)) also develop breathing symptoms, with erratic rhythm and life-threatening apnoeas from postnatal day 30 (P30), leading to respiratory distress and premature death at around P60. We investigated the respiratory function of conscious Mecp2(-/y) mice at P40-P60 using conventional whole-body plethysmography, double-chamber plethysmography and chest EMG recordings. Double-chamber plethysmography revealed a persistent increase in respiratory work-load with enlarged chest movements, but no subsequent increase of tidal volume thus revealing a mismatch between airflow and muscle work-load. Apnoeas occurred with cessation of both chest movements and ventilation, but some (40%) developed with persisting rhythmic chest EMG discharges or chest movements without respiratory airflow, suggesting respiratory efforts against obstructed airways. Airway obstruction was maintained even when the respiratory drive increased significantly, triggering large chest EMG discharges and movements. Whole-body plethysmography of Mecp2(-/y) mice revealed significant increases of spirograms, reflecting forced chest movements against partially obstructed airways. The persisting chest EMG discharges and rhythmic chest movements without respiratory airflow suggest that Mecp2 inactivation alters neural circuits controlling the upper airway dilator muscles. The observed breath-hold events in Mecp2(-/y) mice might imply disturbance of neural circuits attached to voluntary control of breathing.
Affiliation
Mp3-Respiration Team, Centre de Recherche en Neurobiologie et Neurophysiologie de Marseille (CRN2 M), Unité Mixte de Recherche 6231, CNRS - Université Aix-Marseille II & III, Faculté Saint Jérôme, 13397 Marseille Cedex 20, France.
Journal Details
This article was published in the following journal.
Name: Respiratory physiology & neurobiology
ISSN: 1878-1519
Pages: 146-56
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20659592
- DOI: http://dx.doi.org/10.1016/j.resp.2010.07.006
Medical and Biotech [MESH] Definitions
Mice, Inbred Mrl Lpr
A mouse substrain that is genetically predisposed to the development of systemic lupus erythematosus-like syndrome, which has been found to be clinically similar to the human disease. It has been determined that this mouse strain carries a mutation in the fas gene. Also, the MRL/lpr is a useful model to study behavioral and cognitive deficits found in autoimmune diseases and the efficacy of immunosuppressive agents.
Asthma
A form of bronchial disorder with three distinct components: airway hyper-responsiveness (RESPIRATORY HYPERSENSITIVITY), airway INFLAMMATION, and intermittent AIRWAY OBSTRUCTION. It is characterized by spasmodic contraction of airway smooth muscle, WHEEZING, and dyspnea (DYSPNEA, PAROXYSMAL).
Biological Assay
A method of measuring the effects of a biologically active substance using an intermediate in vivo or in vitro tissue or cell model under controlled conditions. It includes virulence studies in animal fetuses in utero, mouse convulsion bioassay of insulin, quantitation of tumor-initiator systems in mouse skin, calculation of potentiating effects of a hormonal factor in an isolated strip of contracting stomach muscle, etc.
Models, Statistical
Statistical formulations or analyses which, when applied to data and found to fit the data, are then used to verify the assumptions and parameters used in the analysis. Examples of statistical models are the linear model, binomial model, polynomial model, two-parameter model, etc.
Airway Remodeling
The structural changes in the number, mass, size and/or composition of the airway tissues.
PubMed Articles
Non-invasive system for applying airway obstructions to model obstructive sleep apnea in mice.
Obstructive sleep apnea (OSA) is characterized by recurrent upper airway obstructions during sleep. The most common animal model of OSA is based on subjecting rodents to intermittent hypoxic exposures...
Upper airway resistance: species-related differences.
In veterinary medicine, upper airway resistance deserves a particular attention in equines athletes and brachycephalic dogs. Due to the anatomical peculiarities of the upper airway and/or pathological...
Disrupted microRNA expression caused by Mecp2 loss in a mouse model of Rett syndrome.
MicroRNAs (miRNAs) are short non-coding RNA molecules that regulate post-transcriptional gene expression. They influence a wide range of physiological functions, including neuronal processes, and are...
Upper airway obstruction resulting from overflexion fixation of the cervical spine is a rare but life-threatening complication after cervical spine surgery. There are few reports of dyspnea after a po...
Airway management in sedated patients.
The decrease in airway muscular tone seen during natural sleep and sedation, as well as in the supine position, can lead to anatomic changes in the structures that surround the airway. Some patients n...
Clinical Trials
Functional Abilities in Rett Syndrome
The purpose of this study is to evaluate and determinate the functional abilities in Rett syndrome conforming to the established Pediatric Evaluation of Disability Inventory (PEDI).
Upper Airway Collapsibility Evaluation in Different Sedative Levels by Sleep Endoscopy
The purpose of this study is to evaluate the upper airway collapsibility by sleep endoscopy in different sedative levels. Propofol pump infusion will be used to achieve conscious sedation....
Upper Airways in Pregnancy: Evaluation by the Acoustic Reflection Method
It is well known that airway management can be difficult during pregnancy. Increased risks for difficult intubation in pregnant women have been often reported. Thus, pregnancy is regarded...
Independent Studies of Dextromethorphan and of Donepezil Hydrochloride for Rett Syndrome
Rett syndrome (RTT) is a disorder in which the nervous system does not develop properly. RTT generally affects girls, but there are some boys who have been diagnosed with RTT. Symptoms of...
Obesity and Asthma:a Specific Phenotype
Clinicians frequently observed that obese women referred for severe asthma do not respond to treatment. These patients, despite the presence of wheezing, often have normal expiratory flows...
