The association of cardiac muscle necrosis and inflammation with the degenerative and persistent myopathy of MDX mice☆
References (5)
Diseases of Muscle — A Study in Pathology
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X Chromosome-linked muscular dystrophy (mdx) in the mouse
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Cardiac CIP protein regulates dystrophic cardiomyopathy
2022, Molecular TherapyMusculoskeletal magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy
2021, Neuromuscular DisordersCitation Excerpt :Beagle crosses (with the same mutation) have been created to develop a separate, smaller line of dogs in Japan, known as CXMDJ [11]. When compared to mdx mice that have minimal clinically applicable musculoskeletal features, canine models more closely reflect the human phenotype, both functionally and histologically [7–9,12–14]. In 2010, we reported a spontaneous (splice site) mutation in a Cavalier King Charles Spaniel that results in deletion of exon 50 and an out of frame transcript [15].
Proteomic analysis of dystrophin deficiency and associated changes in the aged mdx-4cv heart model of dystrophinopathy-related cardiomyopathy
2016, Journal of ProteomicsCitation Excerpt :Low levels of laminin appear to be a key pathophysiological factor in the dystrophic heart [21] that triggers increased whole-organ compliance in Dp427-deficient cardiac muscle [80]. While the interconnectivity within the dystrophin–dystroglycan–laminin axis is weakened, the layers of the interstitial extracellular matrix are increased resulting in fibrotic scarring [57,58,62]. The onset of mdx fibrosis is associated with an increased expression of the connective tissue growth factor CTGF and also elevated levels of one of the main structural classes of proteins forming the extracellular matrix, fibrillar and non-fibrillar collagens [58].
Ibuprofen plus isosorbide dinitrate treatment in the mdx mice ameliorates dystrophic heart structure
2013, Pharmacological ResearchCitation Excerpt :It promotes cardiac muscle excitation contraction coupling [43,44] and organic nitrates are commonly used as treatment for heart failure and stroke [43,45,46]. In addition, cardiomyopathy in DMD shares several aspects of skeletal muscle degeneration, including inflammation [14,43]. These observations prompted us to investigate the potential cardiac benefit of the therapy combining ISDN and IBU; the results we report show that this therapy preserves LV morphology and tended to ameliorate LV function response during stress.
Cardiac phenotype of Duchenne Muscular Dystrophy: Insights from cellular studies
2013, Journal of Molecular and Cellular Cardiology
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This research was supported by the Muscular Dystrophy Group of Great Britain.