Proceedings of the National Academy of Sciences · 2021 · 41 citations · 56 references
Life SciencesBiochemistryMolecular GeneticsAgingMetabolismMitochondrial BiologyMitochondrial DisordersMitochondrial DysfunctionMitochondrial PhysiologyMuscle PhysiologyMuscular DystrophyNeuromuscular ChangesDystrophin DeficiencyMuscle AgingMuscle Fiber DegenerationMuscle Function DeclineMuscle Mass DeclineMuscle Mass MaintenanceMuscular Dystrophy PathophysiologySkeletal Muscle Degeneration
Significance Duchenne muscular dystrophy (DMD) is a fatal degenerative disease without a cure. Current standard pharmacological treatment is corticosteroids. Their prolonged use is associated with several undesirable side effects. Using Caenorhabditis elegans , we have identified pharmacological treatments that supplement hydrogen sulfide (H 2 S). One, sodium GYY4137, largely acts like prednisone to improve neuromuscular health; the other, AP39, targets H 2 S delivery to mitochondria. As these are not steroids, they are unlikely to produce steroid-induced side effects. Additionally, as DMD mice show a decline in total sulfide, our results pave the way for evaluation of cellular and/or mitochondrial H 2 S in DMD pathology and warrant further investigation of selective H 2 S delivery approaches in mdx mice and/or higher animal models of DMD.
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Stochastic and genetic factors influence tissue-specific decline in ageing C. elegans
Laura A. Herndon, Peter J. Schmeissner, Justyna M. Dudaronek et al. · Nature · 2002 · 1.2K citations