Results 1 to 10 of about 7,173 (197)

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 Schneider, Corey Chang, Aneela Altaf, Jennifer Michaels, Mantu Bhaumik, Robert W. Grange, Diego Fraidenraich   +9 more
doaj   +7 more sources

L‐Arginine Activates the Neuregulin‐1/ErbB Receptor Signaling Pathway and Increases Utrophin mRNA Levels in C2C12 Cells [PDF]

Biochemistry Research International, Volume 2025, Issue 1, 2025.
L‐arginine induces the expression of utrophin in skeletal muscle cells, so it has been proposed as a pharmacological treatment to attenuate the symptoms of Duchenne muscular dystrophy (DMD). On the other hand, it has been described that one of the pathways that participates in the expression of utrophin in muscle is the Neuregulin‐1 (NRG‐1)/ErbB ...
Gladys Tapia, Sebastián Fuenzalida, Constanza Rivera, Pía Apablaza, Mónica Silva, Enrique Jaimovich, Nevenka Juretić, Saad Tayyab   +7 more
wiley   +3 more sources

Functional improvement of dystrophic muscle by repression of utrophin: let-7c interaction [PDF]

PLoS ONE, 2017
Duchenne muscular dystrophy (DMD) is a fatal genetic disease caused by an absence of the 427kD muscle-specific dystrophin isoform. Utrophin is the autosomal homolog of dystrophin and when overexpressed, can compensate for the absence of dystrophin and ...
Manoj K. Mishra, Emanuele Loro, Kasturi Sengupta, Steve D. Wilton, Tejvir S. Khurana   +4 more
doaj   +6 more sources

Human dystrophin tandem calponin homology actin‐binding domain crystallized in a closed‐state conformation [PDF]

Acta Crystallographica Section D, Volume 81, Issue 3, Page 122-129, March 2025.
The structure of the N‐terminal actin‐binding domain of human dystrophin was determined, revealing a closed conformation with the first and second calponin homology domains directly interacting.The structure of the N‐terminal actin‐binding domain of human dystrophin was determined at 1.94 Å resolution.
Oakley Streeter, Ke Shi, Joseph Vavra, Hideki Aihara, James M. Ervasti, Robert Evans III, Joseph M. Muretta   +6 more
wiley   +2 more sources

Activation of endogenous full-length utrophin by MyoAAV-UA as a therapeutic approach for Duchenne muscular dystrophy [PDF]

Nature Communications
Activation of endogenous full-length utrophin, a dystrophin homolog, presents an attractive therapeutic strategy for Duchenne muscular dystrophy (DMD), regardless of mutation types and loci.
Ruo Wu, Peng Li, Puhao Xiao, Shu Zhang, Xiaopeng Wang, Jie Liu, Wenjie Sun, Yue Chang, Xiuyi Ai, Lijiao Chen, Yan Zhuo, Jiaojian Wang, Zhengbo Wang, Shangang Li, Yuanyuan Li, Weizhi Ji, Wenting Guo, Shiwen Wu, Yongchang Chen   +18 more
doaj   +2 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, Pao‐Lin Kuo, Patrick C.H. Hsieh   +6 more
openalex   +2 more sources

Daily Treatment with SMTC1100, a Novel Small Molecule Utrophin Upregulator, Dramatically Reduces the Dystrophic Symptoms in the mdx Mouse [PDF]

PLoS ONE, 2011
BACKGROUND:Duchenne muscular dystrophy (DMD) is a lethal, progressive muscle wasting disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the muscle fibers leading to the gradual depletion of skeletal muscle.
Jonathon M. Tinsley, Rebecca J. Fairclough, Richard Storer, Fraser J. Wilkes, Allyson C. Potter, Sarah Squire, Dave Powell, Anna Cozzoli, Roberta Francesca Capogrosso, Adam Lambert, Francis X. Wilson, Stephen P. Wren, Annamaria De Luca, Kay E. Davies   +13 more
openalex   +3 more sources

Functional Substitution by TAT-Utrophin in Dystrophin-Deficient Mice [PDF]

PLoS Medicine, 2009
The loss of dystrophin compromises muscle cell membrane stability and causes Duchenne muscular dystrophy and/or various forms of cardiomyopathy. Increased expression of the dystrophin homolog utrophin by gene delivery or pharmacologic up-regulation has ...
Kevin J. Sonnemann, Hanke Heun-Johnson, Amy Turner, Kristen A. Baltgalvis, Dawn A. Lowe, James M. Ervasti   +5 more
openalex   +3 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

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