Therapeutic Benefits of Nutritional Supplements in Duchenne Muscular Dystrophy

A current paper published in Nutrients journal discusses the potential of various nutritional compounds to improve the progression of Duchenne Muscular Dystrophy (DMD), a mitochondrial myopathic disease.

DMD is a chromosome X-linked genetic disorder and progressive neuromuscular disease that causes muscle weakness, wasting, and cardiac dysfunction. In skeletal muscle dystrophy, mitochondrial dysfunction causes an increase of stress-induced reactive-oxygen species (ROS) (free radical) production. Increased oxidative stress within the cell damages the cell membrane of muscle fiber cells and eventually results in the death of the cell. Muscle fibers undergo an unprogrammed cell death (necrosis) and are ultimately replaced with adipose and connective tissue.

Current therapies for DMD include corticosteroid treatment, which can cause side effects such as high blood sugar, bone and tissue degeneration. Because of an impaired ability to produce ATP (energy), researchers have focused treatments on ways to protect against muscle wasting and regenerate energy-pathways in DMD.

In their paper entitled, Metabogenic and Nutriceutical Approaches to Address Energy Dysregulation and Skeletal Muscle Wasting in Duchenne Muscular Dystrophy, researchers at Victoria University and Western Health in Australia reviewed the use of compounds that support the mitochondria and build energy reserves. Supplements with therapeutic potential include amino acids and protein isolates, due to their role as building blocks and energy reservoirs. Other molecules include creatine (Cr), taurine, glutamine, arginine and whey protein isolates. Another class of supplements include mitochondrial co-factors and modulators, coenzyme Q10, micronutrient compounds, resveratrol, quercetin and epigallocatechin gallate (EGCG).

The researchers reported, “while promising data has been derived using treatment regimens focused on isolated supplements, we suggest that greater therapeutic value could be gained from administering combined adjuvants in supplement regimens designed to target the various deficits and abnormalities evident in the metabolic milieu that regulates skeletal muscle energy balance and the maintenance of functional muscle mass.”