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https://www.readbyqxmd.com/read/29791760/neopterin-7-8-dihydroneopterin-is-elevated-in-duchenne-muscular-dystrophy-patients-and-protects-mdx-skeletal-muscle-function
#1
Angus Lindsay, Alexandra Schmiechen, Christopher M Chamberlain, James M Ervasti, Dawn A Lowe
Macrophage infiltration is a hallmark of dystrophin-deficient muscle. We tested the hypothesis that Duchenne muscular dystrophy (DMD) patients would have elevated levels of the macrophage synthesized pterins, neopterin and 7,8-dihydroneopterin compared to unaffected age-matched controls. Urinary neopterin/creatinine and 7,8-dihydroneopterin/creatinine were elevated in DMD patients and 7,8-dihydroneopterin/creatinine was associated with patient age and ambulation. 7,8-dihydroneopterin correction with specific gravity was also elevated in DMD patients...
May 23, 2018: Experimental Physiology
https://www.readbyqxmd.com/read/29783118/placenta-derived-mesenchymal-stromal-cells-and-their-exosomes-exert-therapeutic-effects-in-duchenne-muscular-dystrophy
#2
Ariel Bier, Peter Berenstein, Noam Kronfeld, Daria Morgoulis, Amotz Ziv-Av, Hodaya Goldstein, Gila Kazimirsky, Simona Cazacu, Rinat Meir, Rachela Popovtzer, Amir Dori, Chaya Brodie
Duchenne muscular dystrophy (DMD) is a degenerative lethal, X-linked disease of skeletal and cardiac muscles caused by mutations in the dystrophin gene. Cell therapy using different cell types, including mesenchymal stromal cells (MSCs), has been considered as a potential approach for the treatment of DMD. MSCs can be obtained from autologous sources such as bone marrow and adipose tissues or from allogeneic placenta and umbilical cord. The safety and therapeutic impact of these cells has been demonstrated in pre-clinical and clinical studies and their functions are attributed to paracrine effects that are mediated by secreted cytokines and extracellular vesicles...
May 3, 2018: Biomaterials
https://www.readbyqxmd.com/read/29774991/aggregate-mesenchymal-stem-cell-delivery-ameliorates-the-regenerative-niche-for-muscle-repair
#3
Marissa A Ruehle, Hazel Y Stevens, Aaron M Beedle, Robert E Guldberg, Jarrod A Call
Duchenne muscular dystrophy (DMD) is a severe muscle wasting disease due to the absence of the dystrophin protein from the muscle cell membrane which renders the muscle susceptible to continuous damage. In DMD patients, muscle weakness, together with cycles of degeneration/regeneration and replacement with non-contractile tissue, limit mobility and lifespan. Since the loss of dystrophin result in loss of polarity and a reduction in the number of self-renewing satellite cells, it is postulated that these patients could achieve an improved quality of life if delivered cells could restore satellite cell function...
May 18, 2018: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/29725254/resveratrol-attenuates-denervation-induced-muscle-atrophy-due-to-the-blockade-of-atrogin-1-and-p62-accumulation
#4
Yuka Asami, Miki Aizawa, Masakazu Kinoshita, Junji Ishikawa, Kunihiro Sakuma
Decrease in activity stress induces skeletal muscle atrophy. A previous study showed that treatment with resveratrol inhibits muscular atrophy in mdx mice, a model of DMD. However, almost all studies using resveratrol supplementation have only looked at adaptive changes in the muscle weight. The present study was designed to elucidate whether the resveratrol-inducing attenuation of skeletal muscle actually reflects the adaptation of muscle fibers themselves, based on the modulation of atrogin-1- or p62-dependent signaling...
2018: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/29723037/celecoxib-treatment-improves-muscle-function-in-mdx-mice-and-increases-utrophin-a-expression
#5
Christine Péladeau, Nadine J Adam, Bernard J Jasmin
Duchenne muscular dystrophy (DMD) is a genetic and progressive neuromuscular disorder caused by mutations and deletions in the dystrophin gene. Although there is currently no cure, one promising treatment for DMD is aimed at increasing endogenous levels of utrophin A to compensate functionally for the lack of dystrophin. Recent studies from our laboratory revealed that heparin treatment of mdx mice activates p38 MAPK, leading to an upregulation of utrophin A expression and improvements in the dystrophic phenotype...
May 3, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29712757/defective-flux-of-thrombospondin-4-through-the-secretory-pathway-impairs-cardiomyocyte-membrane-stability-and-causes-cardiomyopathy
#6
Matthew J Brody, Davy Vanhoutte, Tobias G Schips, Justin G Boyer, Chinmay V Bakshi, Michelle A Sargent, Allen J York, Jeffery D Molkentin
Thrombospondins are stress-inducible secreted glycoproteins with critical functions in tissue injury and healing. Thrombospondin-4 (Thbs4) is protective in cardiac and skeletal muscle where it activates an adaptive endoplasmic reticulum (ER) stress-response, induces expansion of the ER, and enhances sarcolemmal stability. However, it is unclear if Thbs4 has these protective functions from within the cell, from the extracellular matrix, or from the secretion process itself. Here we generated transgenic mice with cardiac-specific overexpression of a secretion-defective mutant of Thbs4 to evaluate its exclusive intracellular and secretion-dependent functions...
April 30, 2018: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/29707417/are-soy-products-effective-in-dmd
#7
Gemma Marston, Steve J Winder
Introduction: In addition to their nutritional value, processed soy bean extracts contain several activities with potential therapeutic benefits. These include anti-oxidants, and tyrosine kinase and protease inhibitory activity. There are also anecdotal reports of health benefits of soy products in alleviating DMD symptoms. Methods: Mdx mice were fed a control soy-free diet or the same diet containing either a proprietary soy preparation (Haelan 951), purified soy isoflavones, purified Bowman-Birk protease inhibitor or a combination of isoflavones and Bowman-Birk inhibitor...
March 27, 2018: PLoS Currents
https://www.readbyqxmd.com/read/29700358/premyogenic-progenitors-derived-from-human-pluripotent-stem-cells-expand-in-floating-culture-and-differentiate-into-transplantable-myogenic-progenitors
#8
Fusako Sakai-Takemura, Asako Narita, Satoru Masuda, Toshifumi Wakamatsu, Nobuharu Watanabe, Takashi Nishiyama, Ken'ichiro Nogami, Matthias Blanc, Shin'ichi Takeda, Yuko Miyagoe-Suzuki
Human induced pluripotent stem cells (hiPSCs) are a potential source for cell therapy of Duchenne muscular dystrophy. To reliably obtain skeletal muscle progenitors from hiPSCs, we treated hiPS cells with a Wnt activator, CHIR-99021 and a BMP receptor inhibitor, LDN-193189, and then induced skeletal muscle cells using a previously reported sphere-based culture. This protocol greatly improved sphere formation efficiency and stably induced the differentiation of myogenic cells from hiPS cells generated from both healthy donors and a patient with congenital myasthenic syndrome...
April 26, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29699580/genetic-deletion-of-muscle-rank-or-selective-inhibition-of-rankl-is-not-as-effective-as-full-length-opg-fc-in-mitigating-muscular-dystrophy
#9
Sébastien S Dufresne, Antoine Boulanger-Piette, Sabrina Bossé, Anteneh Argaw, Dounia Hamoudi, Laetitia Marcadet, Daniel Gamu, Val A Fajardo, Hideo Yagita, Josef M Penninger, A Russell Tupling, Jérôme Frenette
Although there is a strong association between osteoporosis and skeletal muscle atrophy/dysfunction, the functional relevance of a particular biological pathway that regulates synchronously bone and skeletal muscle physiopathology is still elusive. Receptor-activator of nuclear factor κB (RANK), its ligand RANKL and the soluble decoy receptor osteoprotegerin (OPG) are the key regulators of osteoclast differentiation and bone remodelling. We thus hypothesized that RANK/RANKL/OPG, which is a key pathway for bone regulation, is involved in Duchenne muscular dystrophy (DMD) physiopathology...
April 24, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29691609/effect-of-25-hydroxyvitamin-d-deficiency-and-its-interaction-with-prednisone-treatment-on-musculoskeletal-health-in-growing-mdx-mice
#10
Sung-Hee Yoon, Kim S Sugamori, Marc D Grynpas, Jane Mitchell
Duchenne muscular dystrophy (DMD) results from genetic mutations of the gene encoding dystrophin, leading to muscle inflammation and degeneration that is typically treated with glucocorticoids. DMD and its treatment with glucocorticoids result in poor bone health and high risk of fractures. Insufficient levels of 25-hydroxyvitamin D (25-hydroxy D) that may contribute to weakened bone are routinely found in DMD patients. To determine the effect of 25-hydroxy D deficiency, this study examined the effects of low vitamin D dietary intake with and without glucocorticoids on the musculoskeletal system of the Mdx mouse model of DMD...
April 24, 2018: Calcified Tissue International
https://www.readbyqxmd.com/read/29684379/effect-of-a-long-term-treatment-with-metformin-in-dystrophic-mdx-mice-a-reconsideration-of-its-potential-clinical-interest-in-duchenne-muscular-dystrophy
#11
Paola Mantuano, Francesca Sanarica, Elena Conte, Maria Grazia Morgese, Roberta Francesca Capogrosso, Anna Cozzoli, Adriano Fonzino, Angelo Quaranta, Jean-Francois Rolland, Michela De Bellis, Giulia Maria Camerino, Luigia Trabace, Annamaria De Luca
The pharmacological stimulation of AMP-activated protein kinase (AMPK) via metabolic enhancers has been proposed as potential therapeutic strategy for Duchenne muscular dystrophy (DMD). Metformin, a widely-prescribed anti-hyperglycemic drug which activates AMPK via mitochondrial respiratory chain, has been recently tested in DMD patients in synergy with nitric oxide (NO)-precursors, with encouraging results. However, preclinical data supporting the use of metformin in DMD are still poor, and its actions on skeletal muscle appear controversial...
April 20, 2018: Biochemical Pharmacology
https://www.readbyqxmd.com/read/29669436/heme-oxygenase-1-influences-satellite-cells-and-progression-of-duchenne-muscular-dystrophy-in-mice
#12
Katarzyna Pietraszek-Gremplewicz, Magdalena Kozakowska, Iwona Bronisz-Budzynska, Maciej Ciesla, Olga Mucha, Paulina Podkalicka, Magdalena Madej, Urszula Glowniak, Krzysztof Tomasz Szade, Jacek Stepniewski, Mateusz Jez, Kalina Andrysiak, Karolina Bukowska-Strakova, Anna Kaminska, Anna Kostera-Pruszczyk, Alicja Jozkowicz, Agnieszka Loboda, Jozef Dulak
AIMS: Muscle damage in Duchenne Muscular Dystrophy (DMD) caused by the lack of dystrophin is strongly linked to inflammation. Heme oxygenase-1 (HO-1; Hmox1) is an anti-inflammatory and cytoprotective enzyme affecting myoblasts differentiation by inhibiting myomirs. The role of HO-1 has not been so far well addressed in DMD. RESULTS: In dystrophin-deficient mdx mice expression of Hmox1 in limb skeletal muscles and diaphragm is higher than in wild-type animals, being consistently elevated from 8 up to 52 weeks, both in myofibres and inflammatory leukocytes...
April 19, 2018: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29618008/variable-rescue-of-microtubule-and-physiological-phenotypes-in-mdx-muscle-expressing-different-miniaturized-dystrophins
#13
D'anna M Nelson, Angus Lindsay, Luke M Judge, Dongsheng Duan, Jeffrey S Chamberlain, Dawn A Lowe, James M Ervasti
Delivery of miniaturized dystrophin genes via adeno-associated viral vectors is one leading approach in development to treat Duchenne muscular dystrophy. Here we directly compared the functionality of five mini- and micro-dystrophins via skeletal muscle-specific transgenic expression in dystrophin-deficient mdx mice. We evaluated their ability to rescue defects in the microtubule network, passive stiffness, and contractility of skeletal muscle. Transgenic mdx mice expressing the short dystrophin isoform Dp116 served as a negative control...
March 28, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29616985/recording-characteristics-of-impedance-electromyography-needle-electrodes
#14
Hyeuknam Kwon, John F Di Cristina, Seward B Rutkove, Benjamin Sanchez
OBJECTIVE: Needle EMG remains the standard clinical test for neuromuscular disease (NMD) assessment, but it only characterizes myofiber membrane depolarization. On the other hand, electrical impedance provides non-electrically active structural and compositional data of tissues. Here, we designed a prototype of needle electrode integrating impedance and EMG measurement capabilities, the so-called I-EMG needle electrode. APPROACH: We use finite element method models to study the impedance recording characteristics of I-EMG needle electrodes...
April 4, 2018: Physiological Measurement
https://www.readbyqxmd.com/read/29614692/determination-of-qpcr-reference-genes-suitable-for-normalizing-gene-expression-in-a-canine-model-of-duchenne-muscular-dystrophy
#15
John C W Hildyard, Frances Taylor-Brown, Claire Massey, Dominic J Wells, Richard J Piercy
BACKGROUND: Dogs with dystrophin-deficient muscular dystrophy are valuable models of the equivalent human disease, Duchenne Muscular Dystrophy (DMD): unlike the mdx mouse, these animals present a disease severity and progression that closely matches that found in human patients. Canine models are however less thoroughly characterised than the established mdx mouse in many aspects, including gene expression. Analysis of expression in muscle plays a key role in the study of DMD, allowing monitoring and assessment of disease progression, evaluation of novel biomarkers and gauging of therapeutic intervention efficacy...
March 26, 2018: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29601589/influence-of-full-length-dystrophin-on-brain-volumes-in-mouse-models-of-duchenne-muscular-dystrophy
#16
Bauke Kogelman, Artem Khmelinskii, Ingrid Verhaart, Laura van Vliet, Diewertje I Bink, Annemieke Aartsma-Rus, Maaike van Putten, Louise van der Weerd
Duchenne muscular dystrophy (DMD) affects besides muscle also the brain, resulting in memory and behavioral problems. The consequences of dystrophinopathy on gross macroscopic alterations are unclear. To elucidate the effect of full-length dystrophin expression on brain morphology, we used high-resolution post-mortem MRI in mouse models that either express 0% (mdx), 100% (BL10) or a low amount of full-length dystrophin (mdx-XistΔhs). While absence or low amounts of full-length dystrophin did not significantly affect whole brain volume and skull morphology, we found differences in volume of individual brain structures...
2018: PloS One
https://www.readbyqxmd.com/read/29582400/enhancing-endogenous-nitric-oxide-by-whole-body-periodic-acceleration-elicits-neuroprotective-effects-in-dystrophic-neurons
#17
Jose R Lopez, A Uryash, J Kolster, E Estève, R Zhang, J A Adams
We have previously shown that inadequate dystrophin in cortical neurons in mdx mice is associated with age-dependent dyshomeostasis of resting intracellular Ca2+ ([Ca2+ ]i ) and Na+ ([Na+ ]i ), elevated reactive oxygen species (ROS) production, increase in neuronal damage and cognitive deficit. In this study, we assessed the potential therapeutic properties of the whole body periodic acceleration (pGz) to ameliorate the pathology observed in cortical neurons from the mdx mouse. pGz adds small pulses to the circulation, thereby increasing pulsatile shear stress to the vascular endothelium, which in turn increases production of nitric oxide (NO)...
March 26, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29578051/dexpression-patterns-of-regulatory-rnas-including-lncrnas-and-trnas-during-postnatal-growth-of-normal-and-dystrophic-mdx-mouse-muscles-and-their-response-to-taurine-treatment
#18
Lauren C Butchart, Jessica R Terrill, Giulia Rossetti, Robert White, Aleksandra Filipovska, Miranda D Grounds
Post-natal skeletal muscle growth in mice is very rapid and involves complex changes in many cells types over the first 6 weeks of life. The acute onset of dystropathology also occurs around 3 weeks of age in the mdx mouse model of the human disease Duchenne Muscular Dystrophy (DMD). This study investigated (i) alterations in expression patterns of regulatory non-coding RNAs (ncRNAs) in vivo, including miRNAs, lncRNAs and tRNAs, during early growth of skeletal muscles in normal control C57Bl/10Scsn (C57) compared with dystrophic mdx mice from 2-6 weeks of postnatal age, and revealed inherent differences in vivo for levels of 3 ncRNAs between C57 and mdx muscles before the onset of dystropathology...
March 22, 2018: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/29577406/quantifying-myofiber-integrity-using-diffusion-mri-and-random-permeable-barrier-modeling-in-skeletal-muscle-growth-and-duchenne-muscular-dystrophy-model-in-mice
#19
Kerryanne V Winters, Olivier Reynaud, Dmitry S Novikov, Els Fieremans, Sungheon Gene Kim
PURPOSE: To measure the microstructural changes during skeletal muscle growth and progressive pathologies using the random permeable model with diffusion MRI, and compare findings to conventional imaging modalities such as three-point Dixon and T2 imaging. METHODS: In vivo and ex vivo DTI experiments with multiple diffusion times (20-700 ms) were completed on wild-type (n = 22) and muscle-dystrophic mdx mice (n = 8) at various developmental time points. The DTI data were analyzed with the random permeable model framework that provides estimates of the unrestricted diffusion coefficient (D0 ), membrane surface-to-volume ratio (S/V), and membrane permeability (κ)...
March 25, 2018: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/29561896/the-dual-ccr2-ccr5-chemokine-receptor-antagonist-cenicriviroc-reduces-macrophage-infiltration-and-disease-severity-in-duchenne-muscular-dystrophy-dmdmdx-4cv-mice
#20
Feng Liang, Christian Giordano, Dong Shang, Qian Li, Basil J Petrof
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle weakness which is ultimately fatal, most often due to involvement of the diaphragm. Macrophage infiltration of dystrophic muscles has been strongly linked to muscle damage and fibrosis in DMD. We hypothesized that cenicriviroc (CVC), a dual chemokine receptor (CCR2/CCR5) antagonist currently under clinical evaluation for other diseases, could prevent macrophage accumulation and blunt disease progression in the diaphragms of mdx mice (genetic homologue of DMD)...
2018: PloS One
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