β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy

Abstract

Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role in the progression of the pathology. β-glucans can modulate immune function by modifying the phagocytic activity of immunocompetent cells, notably macrophages. Mitochondrial function is also involved in an important mechanism of the innate and adaptive immune responses, owing to high need for energy of immune cells. In the present study, the effects of 1,3-1,6 β-glucans on five-day-old non-dystrophic and dystrophic (sapje) zebrafish larvae were investigated. The effects of the sonication of β-glucans and the dechorionation of embryos were also evaluated. The results showed that the incidence of dystrophic phenotypes was reduced when dystrophic embryos were exposed to 2 and 4 mg L−1 of 1,3-1,6 β-glucans. Moreover, when the dystrophic larvae underwent 8 mg L−1 treatment, an improvement of the locomotor performances and mitochondrial respiration were observed. In conclusion, the observed results demonstrated that 1,3-1,6 β-glucans improve locomotor performances and mitochondrial function in dystrophic zebrafish. Therefore, for ameliorating their life quality, 1,3-1,6 β-glucans look like a promising diet supplement for DMD patients, even though further investigations are required.

Lysosomal Proteomics Links Disturbances in Lipid Homeostasis and Sphingolipid Metabolism to CLN5 Disease

“…Supplementary Table S10: Target sequences and primers used to create morpholino cln5 zebrafish model. References [ 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 ] are cited in the supplementary materials and methods.…”

Potential Health Benefits of Whole Grains: Modulation of Mitochondrial Biogenesis and Energy Metabolism

“…Recently, increasing evidence have indicated that gut microbiome could produce metabolites that influence mitochondrial energy production and mitochondrial biogenesis. − β-Glucan as a kind of dietary fiber has been reported with a beneficial effect on mitochondria metabolism. For instance, β-glucan exposure improved the locomotor performances and mitochondrial respiration in a muscular dystrophic model . In another study, aqueous extracts of fermented barley and β-glucan alleviated high-fat diet (HFD)-induced obesity in rats.…”

“…For instance, β-glucan exposure improved the locomotor performances and mitochondrial respiration in a muscular dystrophic model. 81 In another study, aqueous extracts of fermented barley and β-glucan alleviated high-fat diet (HFD)induced obesity in rats. The authors explained the observed effects by UCP1, thermogenic fat bioenergetics, and mitochondria biosynthesis.…”

Efficient Neuroprotective Rescue of Sacsin-Related Disease Phenotypes in Zebrafish

“…Muscle birefringence, linked to myofibril organization, was analyzed by placing anesthetized embryos on a glass polarizing filter, covering them with a second polarizing filter, and recording them under a Leica M205FA microscope as described elsewhere [84,85]. Embryos were photographed in a bright field.…”