Project 467183

Duchenne Muscular Dystrophy (DMD) is a muscle disease without current cure that results from a defect in the muscle protein dystrophin. Lack of dystrophin makes the muscle fragile and susceptible to damage. DMD primarily affects males and leads to extensive muscle loss and reduced strength. The disease can lead to respiratory failure resulting in premature death in their 20s. Dietary protein is essential to health. The body needs protein to make and repair various cells and tissues in the body such as muscle. Currently, we do not know the required amount of dietary protein for individuals with DMD. Research suggests that increased protein intake may help protect/restore muscle in diseases. Further, not eating enough protein can result in accelerated muscle loss. Our study will be the first to assess protein requirements in boys (12-17 y) with DMD using the Indicator Amino Acid Oxidation (IAAO) technique. The technique uses an indicator amino acid that reveals the amount of protein needed for sufficient protein synthesis. The IAAO technique is non-invasive and safe to use in children and vulnerable groups, as it only requires breath and urine samples following the ingestion of different doses of protein. A total of 8 boys with DMD will undergo 7 sessions of data collection during which they will ingest various amounts of protein ranging from a deficient amount to an excessive amount. During these sessions, breath and urine samples will be collected for estimation of protein requirements. We expect boys with DMD to have a higher protein requirement than that of age-matched controls. This study will establish dietary protein requirements in boys with DMD. Meeting daily protein requirements may affect disease progression and quality of life in a positive way.