Pathophysiological effects of overactive STIM1 on murine muscle function and structure

R Silva-Rojas, AL Charles, S Djeddi, B Geny, J Laporte… - Cells, 2021 - mdpi.com
Store-operated Ca2+ entry (SOCE) is a ubiquitous mechanism regulating extracellular Ca2+
entry to control a multitude of Ca2+-dependent signaling pathways and cellular processes …

Silencing of the Ca2+ Channel ORAI1 Improves the Multi-Systemic Phenotype of Tubular Aggregate Myopathy (TAM) and Stormorken Syndrome (STRMK) in Mice

R Silva-Rojas, L Pérez-Guàrdia, E Lafabrie… - International journal of …, 2022 - mdpi.com
Tubular aggregate myopathy (TAM) and Stormorken syndrome (STRMK) form a clinical
continuum associating progressive muscle weakness with additional multi-systemic …

[HTML][HTML] ORAI1 inhibition as an efficient preclinical therapy for tubular aggregate myopathy and Stormorken syndrome

R Silva-Rojas, L Pérez-Guàrdia, A Simon, S Djeddi… - JCI insight, 2024 - ncbi.nlm.nih.gov
Tubular aggregate myopathy (TAM) and Stormorken syndrome (STRMK) are clinically
overlapping disorders characterized by childhood-onset muscle weakness and a variable …

Valproic acid reduces muscle susceptibility to contraction‐induced functional loss but increases weakness in two murine models of Duchenne muscular dystrophy

D Moutachi, M Lemaitre, C Delacroix… - Clinical and …, 2023 - Wiley Online Library
Skeletal muscles in animal models of Duchenne muscular dystrophy (DMD) are more
susceptible to contraction‐induced functional loss, which is not related to fatigue. Valproic …

Diltiazem improves contractile properties of skeletal muscle in dysferlin‐deficient BLAJ mice, but does not reduce contraction‐induced muscle damage

M Begam, AF Collier, AL Mueller, R Roche… - Physiological …, 2018 - Wiley Online Library
Abstract B6. A‐Dysfprmd/GeneJ (BLAJ) mice model human limb‐girdle muscular dystrophy
2B (LGMD 2B), which is linked to mutations in the dysferlin (DYSF) gene. We tested the …

[HTML][HTML] Damaged muscle fibers might masquerade as hybrid fibers–a cautionary note on immunophenotyping mouse muscle with mouse monoclonal antibodies

M Begam, JA Roche - European Journal of Histochemistry: EJH, 2018 - ncbi.nlm.nih.gov
We report that, labeling mouse muscle tissue, with mouse monoclonal antibodies specific to
slow or fast myosin heavy chain (sMyHC and fMyHC, respectively), can lead to artefactual …

The effects of concentric and eccentric training in murine models of dysferlin‐associated muscular dystrophy

M Begam, R Roche, JJ Hass, CA Basel… - Muscle & …, 2020 - Wiley Online Library
Introduction Dysferlin‐deficient murine muscle sustains severe damage after repeated
eccentric contractions. Methods With a robotic dynamometer, we studied the response of …

Dosage-Adjusted Resistance Training in Mice with a Reduced Risk of Muscle Damage

M Begam, N Narayan, D Mankowski, R Camaj… - JoVE (Journal of …, 2022 - jove.com
Progressive resistance training (PRT), which involves performing muscle contractions
against progressively greater external loads, can increase muscle mass and strength in …

Minimally invasive muscle embedding generates donor-cell-derived muscle fibers that express desmin and dystrophin

JA Roche, M Begam, AK Eaton, CJ Elkins… - Military …, 2020 - academic.oup.com
Introduction The aim of this study was to quantify the extent of donor-cell-derived
myogenesis achieved by a novel surgical technique known as Minimally Invasive Muscle …

HDAC inhibitors as pharmacological treatment for Duchenne muscular dystrophy: a discovery journey from bench to patients

C Mozzetta, V Sartorelli, C Steinkuhler… - Trends in molecular …, 2024 - cell.com
Earlier evidence that targeting the balance between histone acetyltransferases (HATs) and
deacetylases (HDACs), through exposure to HDAC inhibitors (HDACis), could enhance …