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Utrophin upregulation

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https://www.readbyqxmd.com/read/28188344/skeletal-muscle-secretome-in-duchenne-muscular-dystrophy-a-pivotal-anti-inflammatory-role-of-adiponectin
#1
S Lecompte, M Abou-Samra, R Boursereau, L Noel, S M Brichard
BACKGROUND: Persistent inflammation exacerbates the progression of Duchenne muscular dystrophy (DMD). The hormone, adiponectin (ApN), which is decreased in the metabolic syndrome, exhibits anti-inflammatory properties on skeletal muscle and alleviates the dystrophic phenotype of mdx mice. Here, we investigate whether ApN retains its anti-inflammatory action in myotubes obtained from DMD patients. We unravel the underlying mechanisms by studying the secretome and the early events of ApN...
February 10, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28108217/cdk3-is-a-major-target-of-mir-150-in-cell-proliferation-and-anti-cancer-effect
#2
Liang Wang, Yongyong Xi, Chengcao Sun, Feng Zhang, Heng Jiang, Qiqiang He, Dejia Li
MiR-150, a member of small non-coding RNAs, has been proven to dysregulate in different types of tumor and bear on carcinogenesis and cancer prognosis by regulating the expression of a series of gene including utrophin. Given that utrophin can compensate for dystrophin's absence and be regarded as a promising therapeutic target for Duchenne Muscular Dystrophy (DMD), we further detected the deep role of miR-150 in dystrophic muscle. Using a range of bioinformatic, molecular and cell biology techniques, we declared that miR-150 directly targets cyclin-dependent kinase 3 (CDK3) and leads to the regulation of CDK3 gene expression in both muscle-derived and non-muscle cells...
January 17, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/27734557/small-fractions-of-muscular-dystrophy-embryonic-stem-cells-yield-severe-cardiac-and-skeletal-muscle-defects-in-adult-mouse-chimeras
#3
J Patrick Gonzalez, Sergii Kyrychenko, Viktoriia Kyrychenko, Joel S Schneider, Celine J Granier, Eric Himelman, Kevin C Lahey, Qingshi Zhao, Ghassan Yehia, Yuan-Xiang Tao, Mantu Bhaumik, Natalia Shirokova, Diego Fraidenraich
Duchenne muscular dystrophy (DMD) is characterized by the loss of the protein dystrophin, leading to muscle fragility, progressive weakening, and susceptibility to mechanical stress. Although dystrophin-negative mdx mouse models have classically been used to study DMD, phenotypes appear mild compared to patients. As a result, characterization of muscle pathology, especially in the heart, has proven difficult. We report that injection of mdx embryonic stem cells (ESCs) into Wild Type blastocysts produces adult mouse chimeras with severe DMD phenotypes in the heart and skeletal muscle...
October 13, 2016: Stem Cells
https://www.readbyqxmd.com/read/27485975/protein-anchoring-therapy-of-biglycan-for-mdx-mouse-model-of-duchenne-muscular-dystrophy
#4
Mikako Ito, Yuka Ehara, Jin Li, Kosuke Inada, Kinji Ohno
Duchenne muscular dystrophy (DMD) is a devastating muscle disease caused by loss-of-function mutations in DMD encoding dystrophin. No rational therapy is currently available. Utrophin is a paralog of dystrophin and is highly expressed at the neuromuscular junction. In mdx mice, utrophin is naturally upregulated throughout the muscle fibers, which mitigates muscular dystrophy. Protein-anchoring therapy was previously reported, in which a recombinant extracellular matrix (ECM) protein is delivered to and anchored to a specific target using its proprietary binding domains...
August 2, 2016: Human Gene Therapy
https://www.readbyqxmd.com/read/27037492/characterization-of-neuromuscular-synapse-function-abnormalities-in-multiple-duchenne-muscular-dystrophy-mouse-models
#5
Elizabeth M van der Pijl, Maaike van Putten, Erik H Niks, Jan J G M Verschuuren, Annemieke Aartsma-Rus, Jaap J Plomp
Duchenne muscular dystrophy (DMD) is an X-linked myopathy caused by dystrophin deficiency. Dystrophin is present intracellularly at the sarcolemma, connecting actin to the dystrophin-associated glycoprotein complex. Interestingly, it is enriched postsynaptically at the neuromuscular junction (NMJ), but its synaptic function is largely unknown. Utrophin, a dystrophin homologue, is also concentrated at the NMJ, and upregulated in DMD. It is possible that the absence of dystrophin at NMJs in DMD causes neuromuscular transmission defects that aggravate muscle weakness...
June 2016: European Journal of Neuroscience
https://www.readbyqxmd.com/read/26851826/concordant-utrophin-upregulation-in-phenotypically-discordant-dmd-bmd-brothers
#6
Mariz Vainzof, Leticia Feitosa, Marta Canovas, Danielle Ayub-Guerrieri, Rita de Cássia M Pavanello, Mayana Zatz
Utrophin expression was investigated in two phenotypically discordant Duchenne muscular dystrophy half-brothers. The youngest was wheelchair-bound at age 9, while his mildly affected older brother was able to walk without difficulties at age 15. DNA analysis revealed an out-of-frame exon 2 duplication in the DMD gene, associated with muscle dystrophin protein deficiency. Utrophin localization and quantity was analyzed and compared in both sibs to verify whether this could explain the milder phenotype of the older brother...
March 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/26523508/intraperitoneal-injection-of-microencapsulated-sertoli-cells-restores-muscle-morphology-and-performance-in-dystrophic-mice
#7
Sara Chiappalupi, Giovanni Luca, Francesca Mancuso, Luca Madaro, Francesca Fallarino, Carmine Nicoletti, Mario Calvitti, Iva Arato, Giulia Falabella, Laura Salvadori, Antonio Di Meo, Antonello Bufalari, Stefano Giovagnoli, Riccardo Calafiore, Rosario Donato, Guglielmo Sorci
Duchenne muscular dystrophy (DMD) is a genetic disease characterized by progressive muscle degeneration leading to impaired locomotion, respiratory failure and premature death. In DMD patients, inflammatory events secondary to dystrophin mutation play a major role in the progression of the pathology. Sertoli cells (SeC) have been largely used to protect xenogeneic engraftments or induce trophic effects thanks to their ability to secrete trophic, antiinflammatory, and immunomodulatory factors. Here we have purified SeC from specific pathogen-free (SPF)-certified neonatal pigs, and embedded them into clinical grade alginate microcapsules...
January 2016: Biomaterials
https://www.readbyqxmd.com/read/26494902/combinatorial-therapeutic-activation-with-heparin-and-aicar-stimulates-additive-effects-on-utrophin-a-expression-in-dystrophic-muscles
#8
Christine Péladeau, Aatika Ahmed, Adel Amirouche, Tara E Crawford Parks, Lucas M Bronicki, Vladimir Ljubicic, Jean-Marc Renaud, Bernard J Jasmin
Upregulation of utrophin A is an attractive therapeutic strategy for treating Duchenne muscular dystrophy (DMD). Over the years, several studies revealed that utrophin A is regulated by multiple transcriptional and post-transcriptional mechanisms, and that pharmacological modulation of these pathways stimulates utrophin A expression in dystrophic muscle. In particular, we recently showed that activation of p38 signaling causes an increase in the levels of utrophin A mRNAs and protein by decreasing the functional availability of the destabilizing RNA-binding protein called K-homology splicing regulatory protein, thereby resulting in increases in the stability of existing mRNAs...
January 1, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/26257862/involvement-of-adiponectin-in-the-pathogenesis-of-dystrophinopathy
#9
Michel Abou-Samra, Sophie Lecompte, Olivier Schakman, Laurence Noel, Marie C Many, Philippe Gailly, Sonia M Brichard
BACKGROUND: The hormone adiponectin (ApN) is decreased in the metabolic syndrome, where it plays a key pathogenic role. ApN also exerts some anti-inflammatory effects on skeletal muscles in mice exposed to acute or chronic inflammation. Here, we investigate whether ApN could be sufficiently potent to counteract a severe degenerative muscle disease, with an inflammatory component such as Duchenne muscular dystrophy (DMD). METHODS: Mdx mice (a DMD model caused by dystrophin mutation) were crossed with mice overexpressing ApN in order to generate mdx-ApN mice; only littermates were used...
2015: Skeletal Muscle
https://www.readbyqxmd.com/read/26230628/cis-acting-sequence-elements-and-upstream-open-reading-frame-in-mouse-utrophin-a-5-utr-repress-cap-dependent-translation
#10
Trinath Ghosh, Utpal Basu
Utrophin, the autosomal homologue of dystrophin can functionally compensate for dystrophin deficiency. Utrophin upregulation could therefore be a therapeutic strategy in Duchenne Muscular Dystrophy (DMD) that arises from mutation in dystrophin gene. In contrast to its transcriptional regulation, mechanisms operating at post-transcriptional level of utrophin expression have not been well documented. Although utrophin-A 5'-UTR has been reported with internal ribosome entry site (IRES), its inhibitory effect on translation is also evident...
2015: PloS One
https://www.readbyqxmd.com/read/25921779/disease-course-in-mdx-utrophin-mice-comparison-of-three-mouse-models-of-duchenne-muscular-dystrophy
#11
Abby A McDonald, Sadie L Hebert, Matthew D Kunz, Steven J Ralles, Linda K McLoon
The mdx mouse model of Duchenne muscular dystrophy (DMD) is used to study disease mechanisms and potential treatments, but its pathology is less severe than DMD patients. Other mouse models were developed to more closely mimic the human disease based on knowledge that upregulation of utrophin has a protective effect in mdx muscle. An mdx:utrophin(-/-) (dko) mouse was created, which had a severe disease phenotype and a shortened life span. An mdx:utrophin(+/-) mouse was also created, which had an intermediate disease phenotype compared to the mdx and dko mice...
April 2015: Physiological Reports
https://www.readbyqxmd.com/read/25859846/metabolic-dysfunction-and-altered-mitochondrial-dynamics-in-the-utrophin-dystrophin-deficient-mouse-model-of-duchenne-muscular-dystrophy
#12
Meghna Pant, Danesh H Sopariwala, Naresh C Bal, Jeovanna Lowe, Dawn A Delfín, Jill Rafael-Fortney, Muthu Periasamy
The utrophin-dystrophin deficient (DKO) mouse model has been widely used to understand the progression of Duchenne muscular dystrophy (DMD). However, it is unclear as to what extent muscle pathology affects metabolism. Therefore, the present study was focused on understanding energy expenditure in the whole animal and in isolated extensor digitorum longus (EDL) muscle and to determine changes in metabolic enzymes. Our results show that the 8 week-old DKO mice consume higher oxygen relative to activity levels...
2015: PloS One
https://www.readbyqxmd.com/read/25557727/histological-and-biochemical-outcomes-of-cardiac-pathology-in-mdx-mice-with-dietary-quercetin-enrichment
#13
Christopher Ballmann, Katrin Hollinger, Joshua T Selsby, Rajesh Amin, John C Quindry
What is the central question of this study? Does dietary quercetin enrichment improve biochemical and histological outcomes in hearts from mdx mice? What is the main finding and what is its importance? Biochemical and histological findings suggest that chronic quercetin feeding of mdx mice may improve mitochondrial function and attenuate tissue pathology. Patients with Duchenne muscular dystrophy suffer from cardiac pathology, which causes up to 40% of all deaths because of fibrosis and cardiac complications...
January 2015: Experimental Physiology
https://www.readbyqxmd.com/read/25504048/sarcospan-integration-into-laminin-binding-adhesion-complexes-that-ameliorate-muscular-dystrophy-requires-utrophin-and-%C3%AE-7-integrin
#14
Jamie L Marshall, Jennifer Oh, Eric Chou, Joy A Lee, Johan Holmberg, Dean J Burkin, Rachelle H Crosbie-Watson
Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene that result in loss of the dystrophin-glycoprotein complex, a laminin receptor that connects the myofiber to its surrounding extracellular matrix. Utrophin, a dystrophin ortholog that is normally localized to the neuromuscular junction, is naturally upregulated in DMD muscle, which partially compensates for the loss of dystrophin. Transgenic overexpression of utrophin causes broad sarcolemma localization of utrophin, restoration of laminin binding and amelioration of disease in the mdx mouse model of DMD...
April 1, 2015: Human Molecular Genetics
https://www.readbyqxmd.com/read/25442501/systemic-delivery-of-human-bone-marrow-embryonic-like-stem-cells-improves-motor-function-of-severely-affected-dystrophin-utrophin-deficient-mice
#15
Rong-Qing Pang, Jie He, Yong-Yun Zhang, Fu Xiong, Guang-Ping Ruan, Xiang-Qing Zhu, Qiang Wang, Jin-Xiang Wang, Guang-Xu Zhu, Jing Zhao, Xue-Min Cai, Xing-Hua Pan, Cheng Zhang
BACKGROUND AIMS: Embryonic-like stem cells (ELSCs) express embryonic stem cell-specific marker genes, such as SSEA-4, Oct-4 and Nanog, and can be induced to differentiate into cells of all 3 germ layers. Our preliminary data showed that ELSCs isolated from human bone marrow express multipotent antigen markers and differentiate into multinucleated myotube-like cells more efficiently than do mesenchymal stromal cells (MSCs) isolated from the same source. We investigated the therapeutic effect of ELSCs in dystrophin/utrophin double knock-out (dko) mice, one of the Duchenne muscular dystrophy animal models, by systemically transplanting them through tail-vein injection...
December 2014: Cytotherapy
https://www.readbyqxmd.com/read/25324540/utrophin-a-is-essential-in-mediating-the-functional-adaptations-of-mdx-mouse-muscle-following-chronic-ampk-activation
#16
Hasanen Al-Rewashdy, Vladimir Ljubicic, Wei Lin, Jean-Marc Renaud, Bernard J Jasmin
Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin along muscle fibers. An attractive therapeutic avenue for DMD consists in the upregulation of utrophin A, a protein with high sequence identity and functional redundancy with dystrophin. Recent work has shown that pharmacological interventions that induce a muscle fiber shift toward a slower, more oxidative phenotype with increased expression of utrophin A confer morphological and functional improvements in mdx mice. Whether such improvements result from the increased expression of utrophin A per se or are linked to other beneficial adaptations associated with the slow, oxidative phenotype remain to be established...
March 1, 2015: Human Molecular Genetics
https://www.readbyqxmd.com/read/24577720/postanesthetic-death-in-a-cat-with-myopathy
#17
G Remmers, D W Hayden, M A Jaeger, J M Ervasti, S J Valberg
There are few reports of naturally occurring muscular dystrophy in domestic animals. Herein, we describe a case of muscular dystrophy in a 4-year-old neutered male American domestic shorthair cat that died unexpectedly following anesthesia for an elective surgical procedure. Macroscopic muscular hypertrophy and histologic evidence of myofiber size variation, mineralization, myofiber degeneration, and necrosis were compatible with a diagnosis of muscular dystrophy. Extensive endomysial fibrosis was noted histologically in the diaphragm...
January 2015: Veterinary Pathology
https://www.readbyqxmd.com/read/24371285/converging-pathways-involving-microrna-206-and-the-rna-binding-protein-ksrp-control-post-transcriptionally-utrophin-a-expression-in-skeletal-muscle
#18
Adel Amirouche, Helina Tadesse, Pedro Miura, Guy Bélanger, John A Lunde, Jocelyn Côté, Bernard J Jasmin
Several reports have previously highlighted the potential role of miR-206 in the post-transcriptional downregulation of utrophin A in cultured cells. Along those lines, we recently identified K-homology splicing regulator protein (KSRP) as an important negative regulator in the post-transcriptional control of utrophin A in skeletal muscle. We sought to determine whether these two pathways act together to downregulate utrophin A expression in skeletal muscle. Surprisingly, we discovered that miR-206 overexpression in cultured cells and dystrophic muscle fibers causes upregulation of endogenous utrophin A levels...
April 2014: Nucleic Acids Research
https://www.readbyqxmd.com/read/24292612/musculoskeletal-response-of-dystrophic-mice-to-short-term-low-intensity-high-frequency-vibration
#19
S A Novotny, M D Eckhoff, B C Eby, J A Call, D Nuckley, D A Lowe
OBJECTIVES: We aimed to identify parameters of low-intensity vibration that initiate the greatest osteogenic response in dystrophin-deficient mice and determine vibration safety for diseased muscle in three separate studies. METHODS: Study1: Mdx mice were randomized into seven vibration treatments and 14 d later, plasma osteocalcin and tibial osteogenic gene expression were compared among treatments. Study2: Three days of vibration was compared to other modalities known to elicit muscle injury in mdx mice...
December 2013: Journal of Musculoskeletal & Neuronal Interactions
https://www.readbyqxmd.com/read/24145553/vascular-delivery-of-raavrh74-mck-galgt2-to-the-gastrocnemius-muscle-of-the-rhesus-macaque-stimulates-the-expression-of-dystrophin-and-laminin-%C3%AE-2-surrogates
#20
Louis G Chicoine, Louise R Rodino-Klapac, Guohong Shao, Rui Xu, William G Bremer, Marybeth Camboni, Bethannie Golden, Chrystal L Montgomery, Kimberly Shontz, Kristin N Heller, Danielle A Griffin, Sarah Lewis, Brian D Coley, Christopher M Walker, K Reed Clark, Zarife Sahenk, Jerry R Mendell, Paul T Martin
Overexpression of GALGT2 in skeletal muscle can stimulate the glycosylation of α dystroglycan and the upregulation of normally synaptic dystroglycan-binding proteins, some of which are dystrophin and laminin α2 surrogates known to be therapeutic for several forms of muscular dystrophy. This article describes the vascular delivery of GALGT2 gene therapy in a large animal model, the rhesus macaque. Recombinant adeno-associated virus, rhesus serotype 74 (rAAVrh74), was used to deliver GALGT2 via the femoral artery to the gastrocnemius muscle using an isolated focal limb perfusion method...
April 2014: Molecular Therapy: the Journal of the American Society of Gene Therapy
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