6/23/2023 0 Comments Taurine dosageIn these studies, administration of taurine to mdx mice prevented myofibre necrosis, improved both in vivo and ex vivo muscle strength, increased fatigue resistance and decreased other indices of mdx pathology such as inflammation and oxidative stress. The semi-essential amino acid taurine (2-aminoethanesulfonic acid) has been investigated as a pharmacological intervention, using the mdx mouse model of DMD. Consequently, there is considerable interest in developing safe and efficacious therapeutic drug or nutritional interventions that can prevent or slow the progression of DMD (reviewed in ). While much effort has been dedicated to developing genetic or molecular therapies to replace the missing dystrophic protein, there is no cure yet for DMD: the standard drug treatment using corticosteroids is limited in efficacy and associated with severe side effects. The severe loss of muscle mass and function in DMD boys leads to premature death often due to respiratory or cardiac failure (reviewed in ). Consequently, dystrophic muscle undergoes repeated cycles of myofibre necrosis, associated with subsequent inflammation, fibrosis and regeneration: where this damage persists over many months and years in DMD boys it results in the replacement of muscle with fat and fibrotic tissue. DMD is caused by mutations in the dystrophin gene resulting in the loss of functional dystrophin protein in muscle, which leads to increased susceptibility of sarcolemmal damage and necrosis after muscle contraction. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.Ĭompeting interests: The authors have declared that no competing interests exist.ĭuchenne Muscular Dystrophy (DMD) is a lethal, X-chromosome linked muscle disease affecting about 1 in 3500–6000 boys worldwide (Reviewed in ). This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.ĭata Availability: All relevant data are within the paper and its Supporting Information files.įunding: This research was funded by the National Health and Medical Research Council of Australia ( ), grant number APP1065829 (PGA). Received: JAccepted: OctoPublished: November 2, 2017Ĭopyright: © 2017 Terrill et al. PLoS ONE 12(11):Įditor: Jozef Dulak, Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, POLAND Therefore, monitoring of taurine dosage needs to be considered in future pre-clinical trials, in anticipation of using taurine as a clinical therapy for growing DMD boys (and other conditions).Ĭitation: Terrill JR, Pinniger GJ, Nair KV, Grounds MD, Arthur PG (2017) Beneficial effects of high dose taurine treatment in juvenile dystrophic mdx mice are offset by growth restriction. These data caution that a high dose of taurine can have adverse effects and may be less efficacious than lower taurine doses. We present novel data that a high taurine dose increases the cysteine content of both mdx liver and plasma, a possible result of down regulation of the taurine synthesis pathway in the liver (which functions to dispose of excess cysteine, which is toxic). While the dose did decrease inflammation and protein oxidation in dystrophic muscles, there was no improvement in muscle strength (in contrast with benefits observed with the lower dose) and growth of the young mice was significantly restricted. Mice were treated with a dose of taurine estimated to be 16 g/kg/day, in drinking water from 1–6 weeks of age, after which in vivo and ex vivo muscle strength was assessed, as were measures of inflammation, oxidative stress and taurine metabolism. This study aimed to establish if treating growing mdx mice with a higher dose of taurine was more effective at improving strength and reducing inflammation and oxidative stress. We have shown that the amino acid taurine is safe and effective at preventing dystropathology in the mdx mouse model for DMD. Duchenne Muscular Dystrophy (DMD) is a fatal muscle wasting disease manifested in young boys, for which there is no current cure.
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