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https://www.readbyqxmd.com/read/29783118/placenta-derived-mesenchymal-stromal-cells-and-their-exosomes-exert-therapeutic-effects-in-duchenne-muscular-dystrophy
#1
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/29712757/defective-flux-of-thrombospondin-4-through-the-secretory-pathway-impairs-cardiomyocyte-membrane-stability-and-causes-cardiomyopathy
#2
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/29691609/effect-of-25-hydroxyvitamin-d-deficiency-and-its-interaction-with-prednisone-treatment-on-musculoskeletal-health-in-growing-mdx-mice
#3
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
#4
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/29614692/determination-of-qpcr-reference-genes-suitable-for-normalizing-gene-expression-in-a-canine-model-of-duchenne-muscular-dystrophy
#5
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
#6
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
#7
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
#8
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/29556057/self-assembling-asymmetric-peptide-dendrimer-micelles-a-platform-for-effective-and-versatile-in-vitro-nucleic-acid-delivery
#9
Ganesh R Kokil, Rakesh N Veedu, Bao Tri Le, Grant A Ramm, Harendra S Parekh
Despite advancements in the development of high generation cationic-dendrimer systems for delivery of nucleic acid-based therapeutics, commercially available chemical agents suffer from major drawbacks such as cytotoxicity while being laborious and costly to synthesize. To overcome the aforementioned limitations, low-generation cationic peptide asymmetric dendrimers with side arm lipid (cholic and decanoic acid) conjugation were designed, synthesized and systematically screened for their ability to self-assemble into micelles using dynamic light scattering...
March 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29546607/dystrophin-expressing-chimeric-dec-human-cells-provide-a-potential-therapy-for-duchenne-muscular-dystrophy
#10
Maria Siemionow, Joanna Cwykiel, Ahlke Heydemann, Jesus Garcia, Enza Marchese, Krzysztof Siemionow, Erzsebet Szilagyi
Duchenne Muscular Dystrophy (DMD) is a progressive and lethal disease caused by mutations of the dystrophin gene. Currently no cure exists. Stem cell therapies targeting DMD are challenged by limited engraftment and rejection despite the use of immunosuppression. There is an urgent need to introduce new stem cell-based therapies that exhibit low allogenic profiles and improved cell engraftment. In this proof-of-concept study, we develop and test a new human stem cell-based approach to increase engraftment, limit rejection, and restore dystrophin expression in the mdx/scid mouse model of DMD...
June 2018: Stem Cell Reviews
https://www.readbyqxmd.com/read/29484837/elevated-glut4-and-glycogenin-protein-abundance-correspond-to-increased-glycogen-content-in-the-soleus-muscle-of-mdx-mice-with-no-benefit-associated-with-taurine-supplementation
#11
Robert G Barker, Barnaby P Frankish, Hongyang Xu, Robyn M Murphy
Duchenne muscular dystrophy (DMD) patients and the dystrophic mdx mouse have an elevated demand for ATP requiring processes, including Ca2+ regulation and skeletal muscle regeneration. As a key substrate for cellular ATP production, altered glycogen metabolism may contribute significantly to dystrophic pathology and explain reports of mild glucose intolerance. We compare the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse during active muscle necrosis (at 28 days) and at 70 days where pathology is stable...
March 2018: Physiological Reports
https://www.readbyqxmd.com/read/29479480/humanizing-the-mdx-mouse-model-of-dmd-the-long-and-the-short-of-it
#12
REVIEW
Nora Yucel, Alex C Chang, John W Day, Nadia Rosenthal, Helen M Blau
Duchenne muscular dystrophy (DMD) is a common fatal heritable myopathy, with cardiorespiratory failure occurring by the third decade of life. There is no specific treatment for DMD cardiomyopathy, in large part due to a lack of understanding of the mechanisms underlying the cardiac failure. Mdx mice, which have the same dystrophin mutation as human patients, are of limited use, as they do not develop early dilated cardiomyopathy as seen in patients. Here we summarize the usefulness of the various commonly used DMD mouse models, highlight a model with shortened telomeres like humans, and identify directions that warrant further investigation...
2018: NPJ Regenerative Medicine
https://www.readbyqxmd.com/read/29478899/exosome-mediated-benefits-of-cell-therapy-in-mouse-and-human-models-of-duchenne-muscular-dystrophy
#13
Mark A Aminzadeh, Russell G Rogers, Mario Fournier, Rachel E Tobin, Xuan Guan, Martin K Childers, Allen M Andres, David J Taylor, Ahmed Ibrahim, Xiangming Ding, Angelo Torrente, Joshua M Goldhaber, Michael Lewis, Roberta A Gottlieb, Ronald A Victor, Eduardo Marbán
Genetic deficiency of dystrophin leads to disability and premature death in Duchenne muscular dystrophy (DMD), affecting the heart as well as skeletal muscle. Here, we report that clinical-stage cardiac progenitor cells, known as cardiosphere-derived cells (CDCs), improve cardiac and skeletal myopathy in the mdx mouse model of DMD. Injection of CDCs into the hearts of mdx mice augments cardiac function, ambulatory capacity, and survival. Exosomes secreted by human CDCs reproduce the benefits of CDCs in mdx mice and in human induced pluripotent stem cell-derived Duchenne cardiomyocytes...
March 13, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/29477453/normalization-of-connexin-43-protein-levels-prevents-cellular-and-functional-signs-of-dystrophic-cardiomyopathy-in-mice
#14
J Patrick Gonzalez, Jayalakshmi Ramachandran, Eric Himelman, Myriam A Badr, Chifei Kang, Julie Nouet, Nadezhda Fefelova, Lai-Hua Xie, Natalia Shirokova, Jorge E Contreras, Diego Fraidenraich
Duchenne muscular dystrophy (DMD) associated cardiomyopathy remains incurable. Connexin 43 (Cx43) is upregulated and remodeled in the hearts of mdx mice, a mouse model of DMD. Hearts from Wild Type, mdx, and mdx:Cx43(+/-) mice were studied before (4-6 months) and after (10-15 months) the onset of cardiomyopathy to assess the impact of decreasing Cx43 levels on cardiac pathology in dystrophic mice. Increased connexin 43 protein levels in mdx hearts were not observed in mdx:Cx43(+/-) hearts. Cx43 remodeling in mdx hearts was attenuated in mdx:Cx43(+/-) hearts...
April 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29466448/a-dystrophic-duchenne-mouse-model-for-testing-human-antisense-oligonucleotides
#15
Marcel Veltrop, Laura van Vliet, Margriet Hulsker, Jill Claassens, Conny Brouwers, Cor Breukel, Jos van der Kaa, Margot M Linssen, Johan T den Dunnen, Sjef Verbeek, Annemieke Aartsma-Rus, Maaike van Putten
Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disease generally caused by reading frame disrupting mutations in the DMD gene resulting in loss of functional dystrophin protein. The reading frame can be restored by antisense oligonucleotide (AON)-mediated exon skipping, allowing production of internally deleted, but partially functional dystrophin proteins as found in the less severe Becker muscular dystrophy. Due to genetic variation between species, mouse models with mutations in the murine genes are of limited use to test and further optimize human specific AONs in vivo...
2018: PloS One
https://www.readbyqxmd.com/read/29458012/klf10-gene-expression-modulates-fibrosis-in-dystrophic-skeletal-muscle
#16
Joseph X DiMario
Dystrophic skeletal muscle is characterized by fibrotic accumulation of extracellular matrix components that compromise muscle structure, function, and capacity for regeneration. Tissue fibrosis is often initiated and sustained through transforming growth factor-β (TGF-β) signaling, and Krüppel-like factor 10 (KLF10) is an immediate early gene that is transcriptionally activated in response to TGF-β signaling. It encodes a transcriptional regulator that mediates the effects of TGF-β signaling in a variety of cell types...
May 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29433343/cardiac-specific-expression-of-%C3%AE-h2-r15-mini-dystrophin-normalized-all-electrocardiogram-abnormalities-and-the-end-diastolic-volume-in-a-23-month-old-mouse-model-of-duchenne-dilated-cardiomyopathy
#17
Nalinda B Wasala, Jin-Hong Shin, Yi Lai, Yongping Yue, Federica Montanaro, Dongsheng Duan
Heart disease is a major health threat for Duchenne/Becker muscular dystrophy patients and carriers. Expression of a 6-8 kb mini-dystrophin gene in the heart holds promise to change the disease course dramatically. However, the mini-dystrophin gene cannot be easily studied with adeno-associated virus (AAV) gene delivery because the size of the minigene exceeds AAV packaging capacity. Cardiac protection of the ΔH2-R19 minigene was previously studied using the cardiac-specific transgenic approach. Although this minigene fully normalized skeletal muscle force, it only partially corrected electrocardiogram and heart hemodynamics in dystrophin-null mdx mice that had moderate cardiomyopathy...
March 22, 2018: Human Gene Therapy
https://www.readbyqxmd.com/read/29412689/branched-fibers-from-old-fast-twitch-dystrophic-muscles-are-the-sites-of-terminal-damage-in-muscular-dystrophy
#18
Leonit Kiriaev, Sindy Kueh, John W Morley, Kathryn N North, Peter J Houweling, Stewart I Head
A striking pathological feature of dystrophinopathies is the presence of morphologically abnormal branched skeletal muscle fibers. The deterioration of muscle contractile function in Duchenne muscular dystrophy is accompanied by both an increase in number and complexity of these branched fibers. We propose that when number and complexity of branched fibers reaches a critical threshold, "tipping point" the branches in and of themselves are the site of contraction-induced rupture. In the present study, we use the dystrophic mdx mouse and littermate controls to study the pre-diseased dystrophic fast-twitch EDL muscle at 2-3-weeks, the peak myonecrotic phase at 6-9 weeks and finally "old" at 58-112 weeks...
February 7, 2018: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29408692/proteomic-analysis-of-the-sarcolemma-enriched-fraction-from-dystrophic-mdx-4cv-skeletal-muscle
#19
Sandra Murphy, Margit Zweyer, Michael Henry, Paula Meleady, Rustam R Mundegar, Dieter Swandulla, Kay Ohlendieck
The highly progressive neuromuscular disorder dystrophinopathy is triggered by primary abnormalities in the Dmd gene, which causes cytoskeletal instability and loss of sarcolemmal integrity. Comparative organellar proteomics was employed to identify sarcolemma-associated proteins with an altered concentration in dystrophic muscle tissue from the mdx-4cv mouse model of dystrophinopathy. A lectin agglutination method was used to prepare a sarcolemma-enriched fraction and resulted in the identification of 190 significantly changed protein species...
February 2, 2018: Journal of Proteomics
https://www.readbyqxmd.com/read/29364281/a-simple-and-low-cost-assay-for-measuring-ambulation-in-mouse-models-of-muscular-dystrophy
#20
Elizabeth M Gibbs, Rachelle H Crosbie-Watson
Measuring functional outcomes in the treatment of muscular dystrophy is an essential aspect of preclinical testing. The assessment of voluntary ambulation in mouse models is a non-invasive and reproducible activity assay that is directly analogous to measures of patient ambulation such as the 6-minute walk test and related mobility scores. Many common methods for testing mouse ambulation speed and distance are based on the open field test, where an animal's free movement within an arena is measured over time...
December 29, 2017: Journal of Visualized Experiments: JoVE
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