Sharing the load: compensatory pressure generation in the mdx mouse model
Abstract
In Duchenne muscular dystrophy (DMD) patients, respiratory infections throughout life are common due to inadequate airway clearance, and respiratory failure is the primary cause of death (Ishikawa et al. 2011; LoMauro et al. 2015). Evaluations of respiratory muscle function across a range of motor behaviours can provide important information about the underlying neural control of the life-sustaining task of breathing as well as other essential motor functions in healthy and pathological states. Burns and colleagues (2019) recently published a paper in The Journal of Physiology that presents a comprehensive assessment of respiratory muscle function in the mdx mouse model of DMD at 8 weeks of age. The authors’ primary outcome measure was oesophageal pressure, which represents the combined efforts of primarily the diaphragm and, to a lesser extent, other chest wall muscles. In multiple models of neuromuscular impairment, pressure generation is preserved during ventilatory behaviours but impaired during near-maximal expulsive airway clearance behaviours (Greising et al. 2015; Khurram et al. 2018, 2019). Thus, Burns et al. (2019) hypothesized that respiratory muscle weakness is associated with impaired peak pressure generating capacity in mdx mice. One of the major strengths of this paper is their comprehensive analysis of respiratory muscle function; in addition to measurements of oesophageal pressure, they performed diaphragm and external intercostal (EIC) electromyography,…
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