The muscles responsible for breathing are absolutely essential for keeping us alive, but we generally don't think about them when everything is working correctly. Diseases that cause muscle weakness can also affect the breathing muscles, resulting in the need for breathing support from a machine and increasing the risk of pneumonia and other serious chest problems. In severe weakness, this can even lead to death.
Brunel University London is involved in an exciting research project investigating gene therapy for young children affected by a rare, life-limiting muscle disease called X-linked Myotubular Myopathy or XLMTM. The gene therapy programme is showing very positive early results, with large improvements in muscle function in those children who have received the treatment (Audentes Press Release). Breathing muscle strength is an important outcome being measured in the gene therapy study. There has however been very little research in the past into what constitutes 'normal' breathing muscle strength in children under the age of six.
This research project will therefore measure breathing muscle strength in a group of young children with no health problems, so that we have an accurate reference point when considering the results from the XLMTM gene therapy trial. As gene therapy offers great promise for a number of serious muscle conditions, the results from this study will also be of use in other research projects in the future. The study will also give us a greater understanding of how the breathing muscles develop and mature in the first few years of life.
Meet the Principal Investigator(s) for the project
Related Research Group(s)
Human Performance Exercise and Rehabilitation - Researchers from Health and Life Sciences, working in the cardiorespiratory, vascular, neuromuscular and musculoskeletal research areas linked to human performance, health and rehabilitation.
Project last modified 29/06/2021