Extracellular Matrix Proteomics: The mdx-4cv Mouse Diaphragm as a Surrogate for Studying Myofibrosis in Dystrophinopathy

P Dowling, S Gargan, M Zweyer, D Swandulla… - Biomolecules, 2023 - mdpi.com
The progressive degeneration of the skeletal musculature in Duchenne muscular dystrophy
is accompanied by reactive myofibrosis, fat substitution, and chronic inflammation. Fibrotic …

An injury-responsive Rac-to-Rho GTPase switch drives activation of muscle stem cells through rapid cytoskeletal remodeling

AP Kann, M Hung, W Wang, J Nguyen, PM Gilbert… - Cell Stem Cell, 2022 - cell.com
Many tissues harbor quiescent stem cells that are activated upon injury, subsequently
proliferating and differentiating to repair tissue damage. Mechanisms by which stem cells …

MicroRNA-486–dependent modulation of DOCK3/PTEN/AKT signaling pathways improves muscular dystrophy–associated symptoms

MS Alexander, JC Casar, N Motohashi… - The Journal of …, 2014 - Am Soc Clin Investig
Duchenne muscular dystrophy (DMD) is caused by mutations in the gene encoding
dystrophin, which results in dysfunctional signaling pathways within muscle. Previously, we …

Rational engineering of a functional CpG-free ITR for AAV gene therapy

X Pan, Y Yue, M Boftsi, LP Wasala, NT Tran, K Zhang… - Gene therapy, 2022 - nature.com
Inverted terminal repeats (ITRs) are the only wild-type components retained in the genome
of adeno-associated virus (AAV) vectors. To determine whether ITR modification is a viable …

The miR-206/133b cluster is dispensable for development, survival and regeneration of skeletal muscle

T Boettger, S Wüst, H Nolte, T Braun - Skeletal muscle, 2014 - Springer
Background Three different gene clusters code for the muscle-specific miRNAs miR-206,
miR-1 and miR-133a/b. The two miR-1/133a clusters generate identical mature miR-1 and …

SERCA1 overexpression minimizes skeletal muscle damage in dystrophic mouse models

DAG Mázala, SJP Pratt, D Chen… - … of Physiology-Cell …, 2015 - journals.physiology.org
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting
secondary to repeated muscle damage and inadequate repair. Elevations in intracellular …

Environmental enrichment reverses histone methylation changes in the aged hippocampus and restores age-related memory deficits

SJ Morse, AA Butler, RL Davis, IJ Soller, FD Lubin - Biology, 2015 - mdpi.com
A decline in long-term memory (LTM) formation is a common feature of the normal aging
process, which corresponds with abnormal expression of memory-related genes in the aged …

CD38‐NADase is a new major contributor to Duchenne muscular dystrophic phenotype

A de Zélicourt, A Fayssoil… - EMBO molecular …, 2022 - embopress.org
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration.
Two important deleterious features are a Ca2+ dysregulation linked to Ca2+ influxes …

Proteomic identification of markers of membrane repair, regeneration and fibrosis in the aged and dystrophic diaphragm

S Gargan, P Dowling, M Zweyer, M Henry, P Meleady… - Life, 2022 - mdpi.com
Deficiency in the membrane cytoskeletal protein dystrophin is the underlying cause of the
progressive muscle wasting disease named Duchenne muscular dystrophy. In order to …

Increased plasma lipid levels exacerbate muscle pathology in the mdx mouse model of Duchenne muscular dystrophy

N Milad, Z White, AY Tehrani, S Sellers, FMV Rossi… - Skeletal Muscle, 2017 - Springer
Background Duchenne muscular dystrophy (DMD) is caused by loss of dystrophin
expression and leads to severe ambulatory and cardiac function decline. However, the …