ArticlePloS one2013
Ventilatory chemosensory drive is blunted in the mdx mouse model of Duchenne Muscular Dystrophy (DMD).
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 26 citations in OpenAlex.
- Respiratory performance in Duchenne muscular dystrophy: Clinical manifestations and lessons from animal models.Experimental physiology · 2024Review
- Respiratory characterization of a humanized Duchenne muscular dystrophy mouse model.Respiratory physiology & neurobiology · 2024Article
- Breathing in Duchenne muscular dystrophy: translation to therapy.The Journal of physiology · 2022Review
- Improved Bone Quality and Bone Healing of Dystrophic Mice by Parabiosis.Metabolites · 2021Article
- Impact of estrogen deficiency on diaphragm and leg muscle contractile function in female mdx mice.PloS one · 2021Article
- N-acetylcysteine Decreases Fibrosis and Increases Force-Generating Capacity ofAntioxidants (Basel, Switzerland) · 2019Article
- Renal involvement in the pathogenesis of mineral and bone disorder in dystrophin-deficient mdx mouse.The journal of physiological sciences : JPS · 2019Article
- Recovery of respiratory function in mdx mice co-treated with neutralizing interleukin-6 receptor antibodies and urocortin-2.The Journal of physiology · 2018Article
- Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice.Antioxidants (Basel, Switzerland) · 2017Article
- Sensorimotor control of breathing in the mdx mouse model of Duchenne muscular dystrophy.The Journal of physiology · 2017Article
- Sleep Disordered Breathing in Duchenne Muscular Dystrophy.Current neurology and neuroscience reports · 2017Review
- The Effects of Experimental Sleep Apnea on Cardiac and Respiratory Functions in 6 and 18 Month Old Dystrophic (mdx) Mice.PloS one · 2016Article
- Diastolic dysfunction precedes hypoxia-induced mortality in dystrophic mice.Physiological reports · 2015Article
- Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy.Scientific reports · 2015Article
- Current understanding of molecular pathology and treatment of cardiomyopathy in duchenne muscular dystrophy.Molecules (Basel, Switzerland) · 2015Review
- Early right ventricular fibrosis and reduction in biventricular cardiac reserve in the dystrophin-deficient mdx heart.American journal of physiology. Heart and circulatory physiology · 2015Article
- Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options.Therapeutics and clinical risk management · 2015Review
- From innate to adaptive immune response in muscular dystrophies and skeletal muscle regeneration: the role of lymphocytes.BioMed research international · 2014Review
- Muscle atrophy reversed by growth factor activation of satellite cells in a mouse muscle atrophy model.PloS one · 2014Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Duchenne Muscular Dystrophy (DMD) is caused by mutations in the DMD gene resulting in an absence of dystrophin in neurons and muscle. Respiratory failure is the most common cause of mortality and previous studies have largely concentrated on diaphragmatic muscle necrosis and respiratory failure component. Here, we investigated the integrity of respiratory control mechanisms in the mdx mouse model of DMD. Whole body plethysmograph in parallel with phrenic nerve activity recordings revealed a lower respiratory rate and minute ventilation during normoxia and a blunting of the hypoxic ventilatory reflex in response to mild levels of hypoxia together with a poor performance on a hypoxic stress test in mdx mice. Arterial blood gas analysis revealed low PaO2 and pH and high PaCO2 in mdx mice. To investigate chemosensory respiratory drive, we analyzed the carotid body by molecular and functional means. Dystrophin mRNA and protein was expressed in normal mice carotid bodies however, they are absent in mdx mice. Functional analysis revealed abnormalities in Dejours test and the early component of the hypercapnic ventilatory reflex in mdx mice. Together, these results demonstrate a malfunction in the peripheral chemosensory drive that would be predicted to contribute to the respiratory failure in mdx mice. These data suggest that investigating and monitoring peripheral chemosensory drive function may be useful for improving the management of DMD patients with respiratory failure.
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Registered trials
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