Results 61 to 70 of about 7,799 (249)

Micro-utrophin Improves Cardiac and Skeletal Muscle Function of Severely Affected D2/mdx Mice

Molecular Therapy: Methods & Clinical Development, 2018
Duchenne muscular dystrophy (DMD) is an X-linked muscle-wasting disease caused by mutations in the dystrophin gene. DMD boys are wheelchair-bound around 12 years and generally survive into their twenties.
Tahnee L. Kennedy, Simon Guiraud, Ben Edwards, Sarah Squire, Lee Moir, Arran Babbs, Guy Odom, Diane Golebiowski, Joel Schneider, Jeffrey S. Chamberlain, Kay E. Davies   +10 more
doaj   +1 more source

Steric regulation of tandem calponin homology domain actin-binding affinity. [PDF]

, 2019
Tandem calponin homology (CH1-CH2) domains are common actin-binding domains in proteins that interact with and organize the actin cytoskeleton. Despite regions of high sequence similarity, CH1-CH2 domains can have remarkably different actin-binding ...
Bausch, Andreas, Belardi, Brian, Fletcher, Daniel A, Harris, Andrew R, Jreij, Pamela, Shams, Hengameh, Wei, Kathy   +6 more
core   +1 more source

Heregulin‐induced epigenetic regulation of the utrophin‐A promoter [PDF]

FEBS Letters, 2007
Utrophin is the autosomal homolog of dystrophin, the product of the Duchenne's muscular dystrophy (DMD) locus. Utrophin is of therapeutic interest since its over‐expression can compensate dystrophin's absence. Utrophin is enriched at neuromuscular junctions due to heregulin‐mediated utrophin‐A promoter activation.
Basu, Utpal, Gyrd-Hansen, Mads, Baby, Santhosh M, Lozynska, Olga, Krag, Thomas O B, Jensen, Claus J, Frödin, Morten, Khurana, Tejvir S   +7 more
openaire   +4 more sources

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

Dystroglycan versatility in cell adhesion: a tale of multiple motifs [PDF]

, 2010
Dystroglycan is a ubiquitously expressed heterodimeric adhesion receptor. The extracellular a-subunit makes connections with a number of laminin G domain ligands including laminins, agrin and perlecan in the extracellular matrix and the transmembrane b-
A Ivetic, A Sgambato, A Waite, AJ Baines, AM Belkin, AS Yatsenko, AS Yatsenko, Avd Flier, B Casar, B Casar, B Yang, C Anderson, C Anderson, C Batchelor, C Berthier, C Losasso, C Moore, C Vanni, Chris J Moore, CL Batchelor, CL Batchelor, CM Gould, ED Apel, F Sotgia, F Sotgia, F Sotgia, FM Pavalko, GA Rezniczek, GC Dobbins, HJ Spence, HJ Spence, HJ Spence, J Iida, J Kahl, JCR Jones, JL Ilsley, JL Ilsley, JS Poulton, JW Legg, K Iida, K Russo, K Sumita, K Takahashi, K Takahashi, KJ Langenbach, KR Legate, L Fuentes-Mera, L Heiska, M Bartoli, M Bozzi, M Cavaldesi, M Durbeej, M Durbeej, M Ferletta, M Gautam, M James, M Schneider, M Villarreal-Silva, M Weir, ML Oppizzi, NR Smalheiser, NR Smalheiser, O Ibraghimov-Beskrovnaya, O Thompson, O Thompson, R González-Ramírez, RA Williamson, RM Grady, RO Hynes, S Cross, S Yonemura, SA Oak, SH Gee, SJ Winder, SJ Winder, Steve J Winder, T Haenggi, T Muranen, T Rando, V Mirouse, VJ Fincham, W-M Deng, X Yongmin, Y Lai, Y-J Chen, YW Zhou   +85 more
core   +3 more sources

Alternative Splicing: Molecular Mechanisms, Biological Functions, Diseases, and Potential Therapeutic Targets

MedComm, Volume 6, Issue 12, December 2025.
Alternative splicing (AS) expands proteomic diversity and functional complexity in eukaryotes, regulated by spliceosomal components, RNA elements, and epigenetic modifications. Dysregulated AS contributes to diseases, including cancer, neurodegenerative disorders, and cardiovascular conditions, among others. Therapeutic interventions, such as antisense
Zhi‐Min Zhu, Xiao‐Mei Wu, Yan Hu, Xiao‐Lan Bian, Ya‐Qin Wang, Qiong‐Ni Zhu   +5 more
wiley   +1 more source

Actin behavior in bulk cytoplasm is cell cycle regulated in early vertebrate embryos [PDF]

, 2011
The mechanical properties of cells change as they proceed through the cell cycle, primarily owing to regulation of actin and myosin II. Most models for cell mechanics focus on actomyosin in the cortex and ignore possible roles in bulk cytoplasm.
Abe, Agnew, Amano, Belmont, Bendix, Boucrot, Bray, Brieher, Burkel, Chang, Clark, Condeelis, Cytrynbaum, Dao, Desai, Ding, Eggert, Elinson, Ezzell, Gibson, Hara, Kaji, Kane, Kueh, Kunda, Lenart, Loisel, Maddox, Martin, Matsumura, May, Mitchison, Murray, Murray, Niethammer, Pollard, Pollard, Rankin, Riedl, Rosenblatt, Rosenblatt, Schroeder, Taunton, Theriot, Theriot, Valentine, Velarde, Verde, Waterman-Storer, Welch, Woolner, Wühr, Wühr   +52 more
core   +3 more sources

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

Aryl Hydrocarbon Receptor in Health and Disease

MedComm, Volume 6, Issue 11, November 2025.
Based on the structure and ligands of AhR, this review introduces the AhR‐related signaling pathways and their roles in health and diseases. Agonists and antagonists of AhR as well as new strategies for treatment using the microbial–AhR axis are summarized. A prospect was made for the future use of AhR as a therapeutic target.
Haonan Li, Yufeng Fan, Jizheng Liu, Shumin Dong, Bin Wen, Yunfei Zhang, Xiaocui Wang, Xuemei Duan, Ying Hu, Ze Yan, Huifeng Shang, Yukai Jing   +11 more
wiley   +1 more source

Microutrophin expression in dystrophic mice displays myofiber type differences in therapeutic effects.

PLoS Genetics, 2020
Gene therapy approaches for DMD using recombinant adeno-associated viral (rAAV) vectors to deliver miniaturized (or micro) dystrophin genes to striated muscles have shown significant progress.
Glen B Banks, Jeffrey S Chamberlain, Guy L Odom   +2 more
doaj   +1 more source