Results 71 to 80 of about 7,799 (249)

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 J Turner, Kristen A Baltgalvis, Dawn A Lowe, James M Ervasti   +5 more
doaj   +1 more source

Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene [PDF]

Gene Therapy, 2000
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disorder caused by the lack of a subsarcolemmal protein, dystrophin. We have previously shown that the dystrophin-related protein, utrophin is able to compensate for the lack of dystrophin in the mdx mouse, the mouse model for DMD.
Wakefield, P. M., Tinsley, J. M., Wood, M. J. A., Gilbert, R., Karpati, G., Davies, K. E.   +5 more
openaire   +4 more sources

Estrogen‐Related Receptor Alpha Promotes Skeletal Muscle Regeneration and Mitigates Muscular Dystrophy

The FASEB Journal, Volume 39, Issue 19, 15 October 2025.
ERRα promotes muscle regeneration. ERRα drives angiogenic and mitochondrial metabolic gene program in proliferating and differentiating myogenic cells. ERRα also induces myogenic factor genes such as MyoG. Through these pathways ERRα promotes muscle regeneration in the skeletal muscle in acute injury and chronic myopathy.
Thi Thu Hao Nguyen, Ya Xiang Huang, Svitlana Poliakova, Citu Citu, Eira Mann, Danesh H. Sopariwala, Zhongming Zhao, Ashok Kumar, Vihang A. Narkar   +8 more
wiley   +1 more source

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.
Jonathon M Tinsley, Rebecca J Fairclough, Richard Storer, Fraser J Wilkes, Allyson C Potter, Sarah E Squire, Dave S Powell, Anna Cozzoli, Roberta F Capogrosso, Adam Lambert, Francis X Wilson, Stephen P Wren, Annamaria De Luca, Kay E Davies   +13 more
doaj   +1 more source

Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy [PDF]

, 2017
Duchenne muscular dystrophy (DMD) is characterized by muscle degeneration and progressive weakness. There is considerable inter-patient variability in disease onset and progression, which can confound the results of clinical trials.
, Bello, Luca, Gordish-Dressman, Heather, Head, Stewart I., Hoffman, Eric P., Hogarth, Marshall W., Houweling, Peter J., North, Kathryn N., Pegoraro, Elena, Thomas, Kristen C.   +9 more
core   +2 more sources

The cytoskeletal control of B cell receptor and integrin signaling in normal B cells and chronic lymphocytic leukemia

FEBS Letters, Volume 599, Issue 20, Page 2878-2895, October 2025.
In lymphoid organs, antigen recognition and B cell receptor signaling rely on integrins and the cytoskeleton. Integrins act as mechanoreceptors, couple B cell receptor activation to cytoskeletal remodeling, and support immune synapse formation as well as antigen extraction.
Abhishek Pethe, Tanja Nicole Hartmann
wiley   +1 more source

Endogenous bioluminescent reporters reveal a sustained increase in utrophin gene expression upon EZH2 and ERK1/2 inhibition

Communications Biology, 2023
With the long-term goal of finding therapeutic options for Duchenne muscular dystrophy, a mouse model enabling simultaneous visualisation of Dmd and Utrn is described and inhibitors of PRC2 and ERK1/2 identified to increase utrophin expression.
Hannah J. Gleneadie, Beatriz Fernandez-Ruiz, Alessandro Sardini, Mathew Van de Pette, Andrew Dimond, Rab K. Prinjha, James McGinty, Paul M. W. French, Hakan Bagci, Matthias Merkenschlager, Amanda G. Fisher   +10 more
doaj   +1 more source

An analysis of contractile and protrusive cell behaviors at the superficial surface of the zebrafish neural plate

Developmental Dynamics, Volume 254, Issue 10, Page 1115-1132, October 2025.
Abstract Background The forces underlying convergence and internalization of the teleost neural plate remain unknown. To help understand this morphogenesis, we analyzed collective and individual cell behaviors at the superficial surface of the neural plate as internalization begins to form the neural keel in the hindbrain region of the zebrafish embryo.
Claudio Araya, Raegan Boekemeyer, Francesca Farlie, Lauren Moon, Freshta Darwish, Chris Rookyard, Leanne Allison, Gema Vizcay‐Barrena, Roland Fleck, Millaray Aranda, Masa Tada, Jonathan D. W. Clarke   +11 more
wiley   +1 more source

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

Stress exposure in the mdx mouse model of Duchenne muscular dystrophy provokes a widespread metabolic response

The FEBS Journal, Volume 292, Issue 19, Page 5067-5085, October 2025.
Duchenne muscular dystrophy is a severe neuromuscular wasting disease that is caused by a primary defect in dystrophin protein. A targeted mass‐spectrometry‐based metabolomics assay was conducted to identify the impact of stress exposure on the regulation of biological stress pathways in the mdx mouse model of Duchenne muscular dystrophy.
Erynn E. Johnson, James M. Ervasti
wiley   +1 more source