Results 61 to 70 of about 7,107 (213)

Sticky utrophin messages

The Journal of Cell Biology, 2001
![Graphic][1] Utrophin mRNAs bind to actin. On [page 1173][2], Gramolini et al. report that utrophin mRNA is immobilized by binding to an actin-dependent structure. Manipulation of this system may be important for the therapy of Duchenne muscular dystrophy (DMD).
openaire   +2 more sources

Matricellular Protein CCN5 Gene Transfer Ameliorates Cardiac and Skeletal Dysfunction in mdx/utrn (±) Haploinsufficient Mice by Reducing Fibrosis and Upregulating Utrophin Expression

Frontiers in Cardiovascular Medicine, 2022
Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle degeneration due to dystrophin gene mutations. Patients with DMD initially experience muscle weakness in their limbs during adolescence.
Min Ho Song, Jimeen Yoo, Jae Gyun Oh, Hyun Kook, Woo Jin Park, Dongtak Jeong, Dongtak Jeong   +6 more
doaj   +1 more source

Proteomic Profiling of Myofiber Repair Annexins and Their Role in Duchenne Muscular Dystrophy

PROTEOMICS, EarlyView.
ABSTRACT Myofiber regeneration and membrane repair play crucial roles in maintaining the continuous physiological functioning of the neuromuscular system. A swift and efficient repair mechanism enables the rapid restoration of sarcolemmal integrity following cellular impairment in damaged skeletal muscles.
Paul Dowling, Dounia Bouragba, Elisa Negroni, Capucine Trollet, Margit Zweyer, Dieter Swandulla, Kay Ohlendieck   +6 more
wiley   +1 more source

The atomic structure of human dystrophin spectrin‐like repeat 24

Acta Crystallographica Section F, EarlyView.
The atomic structure of human dystrophin spectrin‐like repeat 24 was determined at 2.14 Å resolution.The structure of spectrin‐like repeat 24 of human dystrophin was determined at 2.5 Å effective resolution. The structure exhibits a three‐helix bundle fold, common to all spectrin‐repeat family members, and shares a high degree of homology with existing
Oakley Streeter, Ke Shi, Hannah Bui, Hideki Aihara, James M. Ervasti, Robert Evans III, Joseph M. Muretta   +6 more
wiley   +1 more source

Proteins implicated in muscular dystrophy and cancer are functional constituents of the centrosome

Life Science Alliance, 2022
This study demonstrates that the muscular dystrophy-associated proteins dystrophin, utrophin, dysferlin, and calpain-3 localize to the centrosome and that their absence leads to excess centrosomes, compromised nuclear morphology, impaired centrosome ...
Lilli Winter, Monika Kustermann, Büsra Ernhofer, Harald Höger, Reginald E Bittner, Wolfgang M Schmidt   +5 more
doaj   +1 more source

Neuromuscular and neuromechanical assessments of respiratory performance in the mdx mouse model of Duchenne muscular dystrophy across the natural history of disease

Experimental Physiology, Volume 111, Issue 4, Page 2279-2307, 1 April 2026.
Abstract Duchenne muscular dystrophy (DMD) is a severe life‐limiting X‐linked neuromuscular disorder characterised by progressive skeletal muscle degeneration and respiratory failure. The mdx mouse, lacking dystrophin, is the most widely used preclinical model of DMD, yet the trajectory of respiratory dysfunction in this model remains incompletely ...
Michael N. Maxwell, Christopher G. Wilson, Mai K. Elmallah, Federica Trucco, Ken D. O'Halloran   +4 more
wiley   +1 more source

Bioengineered AAV9 and Optimised Microdystrophin Vectors Augment Phenotypic Rescue in a Murine Model of Duchenne Muscular Dystrophy

Journal of Cellular and Molecular Medicine, Volume 30, Issue 5, March 2026.
ABSTRACT Duchenne muscular dystrophy (DMD) is a severe neuromuscular disorder without an effective cure. Adeno‐associated virus (AAV) based gene therapy has improved dystrophin function, with sub‐optimal clinical outcomes. We reasoned that a combination of rational engineering of AAV9 capsids modified at the post‐translational modification sites ...
Mohankumar B. Senthilkumar, Sanya Sharma, Navaneeth Srinivasan, Anila Varghese, Pratiksha Sarangi, Vijayata Singh, Narendra Kumar, Devyani Yenurkar, Sudip Mukherjee, Sonal Amit, Sameer Bhatia, Santosh K. Misra, Ratna Dua Puri, Jeffrey Chamberlain, Giridhara R. Jayandharan   +14 more
wiley   +1 more source

Repression-free utrophin-A 5'UTR variants.

Molecular biology research communications, 2019
Mutation in the dystrophin gene results Duchenne Muscular Dystrophy (DMD), an X-linked fatal neuromuscular disorder. Dystrophin deficiency can be compensated by upregulation of utrophin, an autosomal homologue of dystrophin. But the expression of utrophin in adults is restricted to myotendinous and neuromuscular junctions.
Malik, Debasish, Basu, Utpal
openaire   +2 more sources

Current Trends in Duchenne Muscular Dystrophy Research and Therapy: 3D Cardiac Modelling

Journal of Cachexia, Sarcopenia and Muscle, Volume 17, Issue 1, February 2026.
ABSTRACT Duchenne muscular dystrophy (DMD), caused by dystrophin deficiency, presents a multifaceted challenge that affects both skeletal muscle function and cardiomyocyte homeostasis, causing progressive degeneration and life‐threatening cardiac complications by adolescence.
Marta Przymuszała, Marta Białobrzeska, Józef Dulak, Urszula Florczyk‐Soluch   +3 more
wiley   +1 more source

Muscle-Specific SIRT1 Gain-of-Function Increases Slow-Twitch Fibers and Ameliorates Pathophysiology in a Mouse Model of Duchenne Muscular Dystrophy [PDF]

, 2013
SIRT1 is a metabolic sensor and regulator in various mammalian tissues and functions to counteract metabolic and age-related diseases. Here we generated and analyzed mice that express SIRT1 at high levels specifically in skeletal muscle.
Chalkiadaki, Angeliki, Guarente, Leonard Pershing, Igarashi, Masaki, Knezevic, Jovana, Nasamu, Armiyaw Sebastian   +4 more
core   +3 more sources