A5034334956- Muscle Physiology and Disorders
- Adipose Tissue and Metabolism
- Ion channel regulation and function
- Exercise and Physiological Responses
- Cardiomyopathy and Myosin Studies
- Cardiovascular Effects of Exercise
- Genetic Neurodegenerative Diseases
- Virus-based gene therapy research
- Parkinson's Disease Mechanisms and Treatments
- Muscle metabolism and nutrition
- Genetics, Aging, and Longevity in Model Organisms
- Nutrition and Health in Aging
- Viral Infectious Diseases and Gene Expression in Insects
- Cardiac electrophysiology and arrhythmias
- Neuroscience and Neural Engineering
- Adenosine and Purinergic Signaling
- Connexins and lens biology
- Medicinal Plant Extracts Effects
- Coenzyme Q10 studies and effects
- Tissue Engineering and Regenerative Medicine
- Ion Channels and Receptors
- Mesenchymal stem cell research
- Nuclear Structure and Function
- Tribology and Wear Analysis
- Lipid metabolism and disorders
Nantes Université
2015-2024
Institut du Thorax
2013-2018
Inserm
2013-2017
Gene Therapy Laboratory
2017
Translational Research in Gene Therapy
2017
Centre National de la Recherche Scientifique
2006-2015
Centre Hospitalier Universitaire de Nantes
2013
Biocat
2006-2010
Holy Spirit University of Kaslik
2008
Abstract Sepsis, or systemic inflammatory response syndrome, is the major cause of critical illness resulting in admission to intensive care units. Sepsis caused by severe infection and associated with mortality 60% cases. Morbidity due sepsis complicated neuromyopathy, patients face long-term disability muscle weakness, energetic dysfunction, proteolysis wasting. These processes are triggered pro-inflammatory cytokines metabolic imbalances aggravated malnutrition drugs. Skeletal...
A few animal models of Duchenne muscular dystrophy (DMD) are available, large ones such as pigs or dogs being expensive and difficult to handle. Mdx (X-linked dystrophy) mice only partially mimic the human disease, with limited chronic lesions muscle weakness. Their small size also imposes limitations on analyses. rat model could represent a useful alternative since rats animals but 10 times bigger than better reflect functional abnormalities observed in DMD patients. Two lines Dmd...
Abstract Background The systemic rotenone model of Parkinson’s disease (PD) accurately replicates many aspects the pathology human PD, especially neurodegeneration substantia nigra and lesions in enteric nervous system (ENS). Nevertheless, precise effects oral on ENS have not been addressed yet. This study was therefore designed to assess a chronic treatment by neurochemical phenotype, gastrointestinal (GI) motility, intestinal epithelial barrier permeability. Methods Male C57BL6N mice...
Abstract Photoactivatable drugs targeting ligand-gated ion channels open up new opportunities for light-guided therapeutic interventions. Photoactivable toxins have the potential to control excitable cell activities with low invasiveness and high spatiotemporal precision. As proof-of-concept, we develop HwTxIV-Nvoc, a UV light-cleavable photoactivatable peptide that targets voltage-gated sodium (Na V ) validate its activity in vitro HEK293 cells, ex vivo brain slices on mice neuromuscular...
Corticosteroids (CS) are standard therapy for the treatment of Duchenne's muscular dystrophy (DMD). Even though they decrease inflammation, have limited efficacy and associated with significant side effects. There is therefore need new protolerogenic treatments to replace CS. Dystrophin-deficient rats (Dmdmdx) closely resemble pathological phenotype DMD patients. We performed first Immunophenotyping Dmdmdx showed leukocyte infiltration in skeletal cardiac muscles, which consisted mostly...
Duchenne Muscular Dystrophy (DMD) is a severe muscle-wasting disease caused by mutations in the DMD gene encoding dystrophin, expressed mainly muscles but also other tissues like retina and brain. Non-progressing cognitive dysfunction occurs 20 to 50% of patients. Furthermore, loss expression Dp427 dystrophin isoform brain mdx mice, most used animal model DMD, leads behavioral deficits thought be linked insufficiencies synaptogenesis channel clustering at synapses. Mdx mice where locomotor...
Valeriana officinalis L. root extracts are traditionally taken for their sedative and anxiolytic properties also used muscle relaxation. Relaxant effects were clearly observed on smooth whereas data skeletal scarce inconsistent. The aim of this study was to assess whether a standardized extract (SE) V. had myorelaxant by decreasing strength and/or neuromuscular tone in mice. Mice received an acute dose SE (2 or 5 g/kg per os) tetrazepam (10 mg/kg ip), standard drug. Thirty minutes later, the...
Aging is associated with a loss of muscle mass and functional capacity. Present study was designed to compare the impact specific dairy proteins on muscular function or without low-intensity physical activity program treadmill in an aged rat model. We investigated effects nutritional supplementation, five days week over 2-month period slow digestible protein, casein fast proteins, whey soluble milk strength locomotor parameters sedentary active Wistar RjHan rats (17-19 months age). An...
Abstract Background Duchenne muscular dystrophy (DMD) is an X-linked inherited disease caused by mutations in the gene encoding dystrophin that leads to a severe and ultimately life limiting muscle-wasting condition. Recombinant adeno-associated vector (rAAV)-based therapy promising, but size of full-length cDNA exceeds packaging capacity rAAV. Alternative or complementary strategies could treat DMD patients are thus needed. Intracellular calcium overload due sarcolemma permeability (SPCa)...
Tight control of skeletal muscle contractile activation is secured by the excitation-contraction (EC) coupling protein complex, a molecular machinery allowing plasma membrane voltage to activity ryanodine receptor Ca2+ release channel in sarcoplasmic reticulum (SR) membrane. This has been shown be intimately linked pannexin-1 (Panx1). We investigated whether prescription drug probenecid, widely used Panx1 blocker, affects signaling, EC coupling, and force. The effect probenecid was tested on...
Duchenne muscular dystrophy (DMD) is an X-linked disease caused by loss-of-function mutations in the dystrophin gene and characterized muscle wasting early mortality. Adeno-associated virus-mediated therapy being investigated as a treatment for DMD. In nonclinical study documented here, we determined effective dose of fordadistrogene movaparvovec, clinical candidate adeno-associated virus serotype 9 vector carrying human mini-dystrophin transgene, after single intravenous injection...
The transient receptor potential vanilloid 1 (TRPV1) belongs to the superfamily of sensory receptors. TRPV1 is a non-selective cation channel permeable Ca2+ that capable detecting noxious heat temperature and acidosis. In skeletal muscles, operates as reticular Ca2+-leak several mutations have been associated with two muscle disorders: malignant hyperthermia (MH) exertional stroke (EHS). Although TRPV1−/− mice available since 2000s, TRPV1’s role in physiology has not thoroughly studied....
Inflammation and oxidative stress are strongly implicated in the pathology of Duchenne muscular dystrophy (DMD), sulphur-containing amino acid taurine ameliorates both decreases dystropathology mdx mouse model for DMD. We therefore further tested as a therapy using dystrophic DMDmdx rats dmd zebrafish models DMD that have more severe dystropathology. However, treatment had little effect on indices these models. While we others previously observed deficiency mice, current study show rat...
In this study, it was shown that adenosine potentiates caffeine‐induced Ca 2+ release. It then proposed the enhancement of release might occur by a direct effect on ryanodine channel or other regulation mechanisms. Furthermore, A 2A receptors may be functional ferret cardiac sarcoplasmic reticulum. Using chemically skinned fibres, experiments were conducted muscle to find out whether and 1 receptor agonists (CCPA CGS 21680) antagonists (DPCPX ZM 241385) affected sensitivity contractile...
The aim of the present study was to investigate direct effects a reactive oxygen species, H<sub>2</sub>O<sub>2</sub>, on contractile function and sarcoplasmic reticulum properties dystrophin-deficient diaphragm using chemically skinned fibers vesicle preparations. results obtained Triton X-100-skinned demonstrate that exposure 1mMH<sub>2</sub>O<sub>2</sub> had similar maximal Ca<sup>2+</sup>-activated tension Ca<sup>2+</sup> sensitivity apparatus in Bl10 <i>mdx</i> mice....
The potential pathogenic role of disturbed Ca2+ homeostasis in Duchenne muscular dystrophy (DMD) remains a complex, unsettled issue. We used muscle fibers isolated from 3-mo-old DMDmdx rats to further investigate the case. Most exhibited no sign trophic or morphology distinction as compared with WT and mitochondria t-tubule membrane networks also showed stringent discrepancy. Under voltage clamp, values for holding current were similar two groups, whereas capacitance larger fibers,...
Click to increase image sizeClick decrease sizeKeywords: lengthening contractionsknee extensorsexternal workergometer
Abstract Duchenne muscular dystrophy (DMD) has as standard pharmacological therapy with corticoisteroids (CS) that decrease inflammation and immune responses present in patients animal models. CS have however limited efficacy important numerous side effects. Therefore, there is a need for new anti-inflammatory pro-tolerogenic treatments could replace or doses of CS. We first assessed the status system dystrophin-deficient rats ( Dmd mdx ) closely reproduce phenotype DMD patients. showed...
The aim of this study was to investigate the effects adenosine on reverse mode Na + /Ca 2+ exchange. In intact ferret cardiac trabeculae, -free contractures were investigated after treating preparations with ryanodine, a sarcoplasmic reticulum Ca -channel inhibitor, and thapsigargin, -pump inhibitor added suppress function. (50–100 nmol/L), deaminase (ADA, 0.1–0.5 U/L), A 1 2A receptor agonists CCPA (3–100 nmol/L) CGS 21680 (25–100 antagonists DPCPX (25 ZM 241385 tested contractures....
Recent studies have demonstrated a new role for Klf10, Krüppel-like transcription factor, in skeletal muscle, specifically relating to mitochondrial function. Thus, it was of interest analyze additional tissues that are highly reliant on optimal function such as the cerebellum and decipher Klf10 functional structural properties this brain region. In vivo (magnetic resonance imaging localized spectroscopy, behavior analysis) vitro (histology, spectroscopy analysis, enzymatic activity)...