Results 11 to 20 of about 7,107 (213)

Translational regulation of utrophin by miRNAs. [PDF]

PLoS ONE, 2011
BackgroundUtrophin is the autosomal homolog of dystrophin, the product of the Duchenne Muscular Dystrophy (DMD) locus. Its regulation is of therapeutic interest as its overexpression can compensate for dystrophin's absence in animal models of DMD.
Utpal Basu, Olga Lozynska, Catherine Moorwood, Gopal Patel, Steve D Wilton, Tejvir S Khurana   +5 more
doaj   +4 more sources

Utrophin Compensates dystrophin Loss during Mouse Spermatogenesis [PDF]

Scientific Reports, 2017
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder resulting from mutations in the dystrophin gene. The mdx/utrn −/− mouse, lacking in both dystrophin and its autosomal homologue utrophin, is commonly used to model the clinical symptoms of
Hung-Chih Chen, Yu-Feng Chin, David J. Lundy, Chung-Tiang Liang, Ya-Hui Chi, Paolin Kuo, Patrick C. H. Hsieh   +6 more
doaj   +3 more sources

Muscle structure influences utrophin expression in mdx mice. [PDF]

PLoS Genetics, 2014
Duchenne muscular dystrophy (DMD) is a severe muscle wasting disorder caused by mutations in the dystrophin gene. To examine the influence of muscle structure on the pathogenesis of DMD we generated mdx4cv:desmin double knockout (dko) mice.
Glen B Banks, Ariana C Combs, Guy L Odom, Robert J Bloch, Jeffrey S Chamberlain   +4 more
doaj   +4 more sources

Direct AMPK Activation Confers Mutation-Independent Therapeutic Benefit in Duchenne Muscular Dystrophy. [PDF]

J Cachexia Sarcopenia Muscle
ABSTRACT Background Duchenne muscular dystrophy (DMD) is a severe, life‐limiting neuromuscular disorder (NMD) characterized by progressive muscle wasting and mitochondrial dysfunction. Although gene therapies offer promise, even those already approved by regulatory agencies, their use remains constrained by mutation specificity, delivery challenges and
Ng SY, Mikhail AI, Mattina SR, Lesinski MA, Rebalka IA, Hamstra SI, Xhuti D, Fajardo VA, Tarnopolsky MA, Nederveen JP, Steinberg GR, Hawke TJ, Ljubicic V.   +12 more
europepmc   +2 more sources

Respiratory pathology in the mdx/utrn -/- mouse: A murine model for Duchenne Muscular Dystrophy (DMD). [PDF]

PLoS ONE
Duchenne muscular dystrophy (DMD) is an X-linked devastating disease caused by a lack of dystrophin which results in progressive muscle weakness. As muscle weakness progresses, respiratory insufficiency and hypoventilation result in significant morbidity
Marán Y Hernández Rodríguez, Debolina D Biswas, Aoife D Slyne, Jane Lee, Evelyn Scarrow, Sarra M Abdelbarr, Heather Daniels, Ken D O'Halloran, Leonardo F Ferreira, Charles A Gersbach, Mai K ElMallah   +10 more
doaj   +2 more sources

Myonuclear Domain-Associated and Central Nucleation-Dependent Spatial Restriction of Dystrophin Protein Expression. [PDF]

J Cachexia Sarcopenia Muscle
ABSTRACT Background The restoration of uniformly distributed dystrophin protein expression is an important consideration for the development of advanced therapeutics for Duchenne muscular dystrophy (DMD). Methods We have generated a novel genetic mouse model (mdx52‐XistΔhs) that expresses variable and nonuniformly distributed dystrophin protein from ...
Chwalenia K, Feng VY, Hemmer N, Hildyard JCW, Roskrow LE, Piercy RJ, Wang ET, Aartsma-Rus A, van Putten M, Wood MJA, Roberts TC.   +10 more
europepmc   +2 more sources

Promising therapeutic approaches of utrophin replacing dystrophin in the treatment of Duchenne muscular dystrophy

Fundamental Research, 2022
Duchenne muscular dystrophy (DMD) is a serious genetic neuromuscular rare disease that is prevalent and caused by the mutation/deletion of the X-linked DMD gene that encodes dystrophin.
Ruo Wu, Yafeng Song, Shiwen Wu, Yongchang Chen   +3 more
doaj   +1 more source

Cis-Acting Sequence Elements and Upstream Open Reading Frame in Mouse Utrophin-A 5'-UTR Repress Cap-Dependent Translation. [PDF]

PLoS ONE, 2015
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.
Trinath Ghosh, Utpal Basu
doaj   +1 more source

Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice. [PDF]

PLoS ONE, 2011
Duchenne muscular dystrophy (DMD) is an incurable degenerative muscle disorder. We injected WT mouse induced pluripotent stem cells (iPSCs) into mdx and mdx∶utrophin mutant blastocysts, which are predisposed to develop DMD with an increasing degree of ...
Amanda J Beck, Joseph M Vitale, Qingshi Zhao, Joel S Schneider, Corey Chang, Aneela Altaf, Jennifer Michaels, Mantu Bhaumik, Robert Grange, Diego Fraidenraich   +9 more
doaj   +1 more source

Drug discovery for Duchenne muscular dystrophy via utrophin promoter activation screening. [PDF]

PLoS ONE, 2011
Duchenne muscular dystrophy (DMD) is a devastating muscle wasting disease caused by mutations in dystrophin, a muscle cytoskeletal protein. Utrophin is a homologue of dystrophin that can functionally compensate for its absence when expressed at increased
Catherine Moorwood, Olga Lozynska, Neha Suri, Andrew D Napper, Scott L Diamond, Tejvir S Khurana   +5 more
doaj   +1 more source