Results 1 to 10 of about 7,107 (213)

Skeletal muscle‐specific PGC‐1α‐b overexpression prevents eccentric contraction‐induced muscle injury through an utrophin‐independent pathway in mice [PDF]

Physiological Reports
Slower oxidative fibers are more resistant to eccentric contraction (ECC)‐induced muscle damage than fast‐twitch glycolytic fibers, but the mechanisms remain unclear. This study investigated the roles of the exercise‐inducible PGC‐1α isoform PGC‐1α‐b and
Azuma Naito, Nao Tokuda, Nao Yamauchi, Ayaka Niibori, Kazuma Okada, Koichi Himori, Yuki Ashida, Takashi Yamada   +7 more
doaj   +3 more sources

Systemic MyoAAV-saRNA delivery activates endogenous utrophin and rescues dystrophic pathology in mdx mice [PDF]

Regenerative Therapy
Background: Therapeutic activation of endogenous utrophin, a homolog of dystrophin, represents a mutation-agnostic therapeutic strategy with considerable potential for treating Duchenne muscular dystrophy (DMD).
Zhikai Xiahou, Tianyuan Wang, Zilian Zhang, Na Li, Hong Xiao, Zhuo Li, Yanming Gan, Xinwei Hou, Yafeng Song   +8 more
doaj   +2 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   +2 more
exaly   +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

Dystrophin Restorative and Compensatory Gene Addition Therapies for Duchenne Muscular Dystrophy: Could CRISPRa Provide a Realistic Alternative? [PDF]

Muscles
Duchenne muscular dystrophy (DMD), which results from mutations that disrupt the expression of dystrophin proteins, is characterized by progressive muscle fiber wasting and the development of skeletal muscle fibrosis.
Zakaria Rostamitehrani, Rida Javed, Linda Popplewell   +2 more
doaj   +2 more sources

Genome Editing-Mediated Utrophin Upregulation in Duchenne Muscular Dystrophy Stem Cells

Molecular Therapy - Nucleic Acids, 2020
Utrophin upregulation is considered a promising therapeutic strategy for Duchenne muscular dystrophy (DMD). A number of microRNAs (miRNAs) post-transcriptionally regulate utrophin expression by binding their cognate sites in the 3′ UTR.
Kasturi Sengupta, Manoj K Mishra, Emanuele Loro   +2 more
exaly   +3 more sources

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

Biochemistry Research International
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).
Gladys Tapia, Sebastián Fuenzalida, Constanza Rivera, Pía Apablaza, Mónica Silva, Enrique Jaimovich, Nevenka Juretić   +6 more
doaj   +2 more sources

Biallelic variants in the UTRN gene cause a novel form of multiple congenital arthrogryposis [PDF]

Frontiers in Genetics
Arthrogryposis multiplex congenita (AMC) is a large group of congenital conditions characterized by joint contractures affecting two or more body areas.
Evgeniya Melnik, Daria Akimova, Tatiana Markova, Eugene Tatarskiy, Anna Tvorogova, Viktoria Zabnenkova, Vladimir Kenis, Olga Agranovich, Mikhail Skoblov, Elena Dadali   +9 more
doaj   +2 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 ...
Amy J Turner, Hanke Heun-Johnson, Dawn A Lowe   +2 more
exaly   +2 more sources

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

PLoS ONE, 2011
BackgroundDuchenne 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.
Annamaria De Luca
exaly   +3 more sources