N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis

Terrill, Jessica R.; Radley‐Crabb, Hannah G.; Grounds, Miranda D.; Arthur, Peter G.

doi:10.1016/j.nmd.2011.11.007

Cited by 77 publications

(96 citation statements)

References 41 publications

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“…All mice completed 30 min of horizontal treadmill running at 12 m/min, which has been found to increase the extent of dystropathology. 33,34 Immediately following exercise, mice were injected with 1% EBD (Sigma, St. Louis, Missouri) (w/v) in phosphate buffered saline (PBS) (pH 7.5) by intraperitoneal injection at a dose of 100 μL per 10 g per body weight. 35 Prior to injection, the solution was sterilized by passage through a Millex-GP 0.22 μm filter (Milipore, Bedford, Massachusetts) and stored at 4…”

Section: Introductionmentioning

confidence: 99%

Identification of muscle necrosis in the mdx mouse model of Duchenne muscular dystrophy using three-dimensional optical coherence tomography

et al. 2011

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Abstract. Three-dimensional optical coherence tomography (3D-OCT) was used to image the structure and pathology of skeletal muscle tissue from the treadmill-exercised mdx mouse model of human Duchenne muscular dystrophy. Optical coherence tomography (OCT) images of excised muscle samples were compared with coregistered hematoxylin and eosin-stained and Evans blue dye fluorescence histology. We show, for the first time, structural 3D-OCT images of skeletal muscle dystropathology well correlated with co-located histology. OCT could identify morphological features of interest and necrotic lesions within the muscle tissue samples based on intrinsic optical contrast. These findings demonstrate the utility of 3D-OCT for the evaluation of small-animal skeletal muscle morphology and pathology, particularly for studies of mouse models of muscular dystrophy.C 2011 Society of Photo-Optical Instrumentation Engineers (SPIE).

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Section: Introductionmentioning

confidence: 99%

Identification of muscle necrosis in the mdx mouse model of Duchenne muscular dystrophy using three-dimensional optical coherence tomography

et al. 2011

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“…Additionally, in some murine models of Alzheimer disease, the administration of NAC buffered oxidative damage 21 . In an animal model of Duchenne muscular dystrophy, the treatment with NAC significantly decreased the oxidation of GSH and protein thiols, and enhanced muscle protein thiol content 22 .…”

Section: Discussionmentioning

confidence: 94%

In vitro effect of N-acetyl-L-cysteine on glutathione and sulfhydryl levels in X-linked adrenoleukodystrophy patients

Marchetti¹,

Donida²,

Deon³

et al. 2017

Clin. Biomed. Res.

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“…These improvements in membrane integrity resulted in a 47 % decrease in stretch-induced damage [128]. Interestingly, a high dose was able to protect against exercise-induced damage and necrosis after only one week of treatment [449]. Improvements in Ca 2+ handling were also seen in the heart [450].…”

Section: Free Radicals/rosmentioning

confidence: 99%

Pharmacologically Targeting the Primary Defect and Downstream Pathology in Duchenne Muscular Dystrophy

Fairclough

Perkins²,

Davies³

2012

CGT

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DMD is a devastatingly progressive muscle wasting disorder of childhood that significantly shortens life expectancy. Despite efforts to develop an effective therapy that dates back over a century, clinical interventions are still restricted to management of symptoms rather than a cure. The rationale to develop effective therapies changed in 1986 with the discovery of the dystrophin gene. Since then extensive research into both the molecular basis and pathophysiology of DMD has paved the way not only for development of strategies which aim to correct the primary defect, but also towards the identification of countless therapeutic targets with the potential to alleviate the downstream pathology. In addition to gene and cell-based therapies, which aim to deliver the missing gene and/or protein, an exciting spectrum of pharmacological approaches aimed at modulating therapeutic targets within DMD muscle cells through the use of small drugs are also being developed. This review presents promising pharmacological approaches aimed at targeting the primary defect, including suppression of nonsense mutations and functional compensation by upregulation of the dystrophin homologue, utrophin. Downstream of the primary membrane fragility, inflammation and fibrosis are reduced by blocking NF-κB, TGF-α and TGF-β, and free radical damage has been targeted using antioxidants and dietary/nutritional supplements. There are new hopes that ACE and PDE5 inhibitors can protect against skeletal as well as cardiac pathology, and modulating Ca2+ influx, NO, BMP, protein degradation and the mitochondrial permeability pore hold further promise in tackling the complex pathogenesis of this multifaceted disorder.

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N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis

Cited by 77 publications

References 41 publications

Identification of muscle necrosis in the mdx mouse model of Duchenne muscular dystrophy using three-dimensional optical coherence tomography

Identification of muscle necrosis in the mdx mouse model of Duchenne muscular dystrophy using three-dimensional optical coherence tomography

In vitro effect of N-acetyl-L-cysteine on glutathione and sulfhydryl levels in X-linked adrenoleukodystrophy patients

Pharmacologically Targeting the Primary Defect and Downstream Pathology in Duchenne Muscular Dystrophy

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