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 …
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
Many tissues harbor quiescent stem cells that are activated upon injury, subsequently
proliferating and differentiating to repair tissue damage. Mechanisms by which stem cells …
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
Duchenne muscular dystrophy (DMD) is caused by mutations in the gene encoding
dystrophin, which results in dysfunctional signaling pathways within muscle. Previously, we …
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 …
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 …
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
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting
secondary to repeated muscle damage and inadequate repair. Elevations in intracellular …
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
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 …
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 …
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
Deficiency in the membrane cytoskeletal protein dystrophin is the underlying cause of the
progressive muscle wasting disease named Duchenne muscular dystrophy. In order to …
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
Background Duchenne muscular dystrophy (DMD) is caused by loss of dystrophin
expression and leads to severe ambulatory and cardiac function decline. However, the …
expression and leads to severe ambulatory and cardiac function decline. However, the …