Results 61 to 70 of about 7,173 (197)

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

The FEBS Journal, EarlyView.
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. We demonstrated a broad shift in the circulating stress‐relevant plasma metabolome associated with stressful scruff handling that was ...
Erynn E. Johnson, James M. Ervasti
wiley   +1 more source

Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse [PDF]

, 2012
Loss of dystrophin protein due to mutations in the DMD gene causes Duchenne muscular dystrophy. Dystrophin loss also leads to the loss of the dystrophin glycoprotein complex (DGC) from the sarcolemma which contributes to the dystrophic phenotype ...
Andrew Mitchell, Andrä, Assereto, Batchelor, Bonuccelli, Bonuccelli, Chris J. Moore, Clerk, Cluchague, Cox, Deconinck, Dominic J. Wells, Ervasti, Ervasti, Ervasti, Foster, Gaynor Miller, Gazzerro, Goyenvalle, Grady, Greenberg, Gundesli, Hoyte, Hu, Ilsley, James, Judge, Karpati, Konieczny, Lipscomb, Matsumura, McArthur, Miller, Moore, Morgan, Nguyen, Otte, Pereboev, Pichavant, Pulkkinen, Rebecca Terry, Rezniczek, Robert Piggott, Schröder, Sharp, Smith, Sotgia, Sotgia, Steve J. Winder, T. Neil Dear, Thompson, Thompson, Tinsley, Tinsley, Tracy La Riviere, Winder   +55 more
core   +2 more sources

Astrocyte proliferation in the hippocampal dentate gyrus is suppressed across the lifespan of dystrophin‐deficient mdx mice

Experimental Physiology, Volume 110, Issue 4, Page 585-598, 1 April 2025.
Abstract Absence of the structural protein, dystrophin, results in the neuromuscular disorder Duchenne Muscular Dystrophy (DMD). In addition to progressive skeletal muscle dysfunction, this multisystemic disorder can also result in cognitive deficits and behavioural changes that are likely to be consequences of dystrophin loss from central neurons and ...
Kimberley A. Stephenson, Polly Peters, Mark G. Rae, Dervla O'Malley   +3 more
wiley   +1 more source

Metabolic remodeling of dystrophic skeletal muscle reveals biological roles for dystrophin and utrophin in adaptation and plasticity

Molecular Metabolism, 2021
Objectives: Preferential damage to fast, glycolytic myofibers is common in many muscle-wasting diseases, including Duchenne muscular dystrophy (DMD).
Justin P. Hardee, Karen J.B. Martins, Paula M. Miotto, James G. Ryall, Stefan M. Gehrig, Boris Reljic, Timur Naim, Jin D. Chung, Jen Trieu, Kristy Swiderski, Ashleigh M. Philp, Andrew Philp, Matthew J. Watt, David A. Stroud, Rene Koopman, Gregory R. Steinberg, Gordon S. Lynch   +16 more
doaj  

New Horizons in Myotonic Dystrophy Type 1: Cellular Senescence as a Therapeutic Target

BioEssays, Volume 47, Issue 3, March 2025.
Cellular senescence accumulates in the skeletal muscle of patients with myotonic dystrophy Type 1, leading to the production of senescence‐associated secretory phenotype (SASP) factors that have detrimental effects. Targeting these senescent cells with senotherapeutics could restore muscle homeostasis and slow disease progression.
Cécilia Légaré, J. Andrew Berglund, Elise Duchesne, Nicolas A. Dumont   +3 more
wiley   +1 more source

Identification of new dystroglycan complexes in skeletal muscle. [PDF]

PLoS ONE, 2013
The dystroglycan complex contains the transmembrane protein β-dystroglycan and its interacting extracellular mucin-like protein α-dystroglycan. In skeletal muscle fibers, the dystroglycan complex plays an important structural role by linking the ...
Eric K Johnson, Bin Li, Jung Hae Yoon, Kevin M Flanigan, Paul T Martin, James Ervasti, Federica Montanaro   +6 more
doaj   +1 more source

Drp2 and Periaxin Form Cajal Bands with Dystroglycan But Have Distinct Roles in Schwann Cell Growth [PDF]

, 2012
Cajal bands are cytoplasmic channels flanked by appositions where the abaxonal surface of Schwann cell myelin apposes and adheres to the overlying plasma membrane.
Brophy, Peter J., Gillespie, C. Stewart, Grove, Matthew, Sherman, Diane L., Wu, Lai Man N.   +4 more
core   +1 more source

Salivary Proteome Is Altered in Children With Small Area Thermal Burns

PROTEOMICS – Clinical Applications, Volume 19, Issue 2, March 2025.
ABSTRACT Saliva is a child appropriate biofluid, but it has not previously been used to evaluate the systemic response to burn injury in children. The aim of this study was to investigate the salivary proteome of children with small area thermal skin burns relative to different burn characteristics (mechanism, time to re‐epithelialization and risk of ...
Morgan Carlton, Tuo Zang, Tony J. Parker, Chamindie Punyadeera, Joanne Voisey, Leila Cuttle   +5 more
wiley   +1 more source

Voltage-gated ion channel dysfunction precedes cardiomyopathy development in the dystrophic heart. [PDF]

PLoS ONE, 2011
Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin gene, is associated with severe cardiac complications including cardiomyopathy and cardiac arrhythmias.
Xaver Koenig, Sandra Dysek, Stefanie Kimbacher, Agnes K Mike, Rene Cervenka, Peter Lukacs, Katrin Nagl, Xuan B Dang, Hannes Todt, Reginald E Bittner, Karlheinz Hilber   +10 more
doaj   +1 more source

The utrophin actin-binding domain binds F-actin in two different modes: implications for the spectrin superfamily of proteins [PDF]

, 2002
Utrophin, like its homologue dystrophin, forms a link between the actin cytoskeleton and the extracellular matrix. We have used a new method of image analysis to reconstruct actin filaments decorated with the actin-binding domain of utrophin, which ...
Egelman, Edward H., Ervasti, James M., Galkin, Vitold E., Orlova, Albina, Rybakova, Inna N., VanLoock, Margaret S.   +5 more
core   +2 more sources