A5042834360- Cellular transport and secretion
- Skin and Cellular Biology Research
- Cardiac Imaging and Diagnostics
- Cardiovascular Function and Risk Factors
- Caveolin-1 and cellular processes
- Erythrocyte Function and Pathophysiology
- RNA Interference and Gene Delivery
- Antimicrobial Peptides and Activities
- Virus-based gene therapy research
- Cellular Mechanics and Interactions
- Genetic Neurodegenerative Diseases
- CAR-T cell therapy research
- Muscle Physiology and Disorders
- Cardiovascular Disease and Adiposity
- Viral Infectious Diseases and Gene Expression in Insects
- Cardiomyopathy and Myosin Studies
- Neurogenetic and Muscular Disorders Research
- Advanced biosensing and bioanalysis techniques
- Lipid Membrane Structure and Behavior
- Ion channel regulation and function
- Inflammatory Myopathies and Dermatomyositis
- CRISPR and Genetic Engineering
- Polydiacetylene-based materials and applications
- Cardiac electrophysiology and arrhythmias
- Chemotherapy-induced cardiotoxicity and mitigation
Inserm
2008-2024
Sorbonne Université
2018-2024
Institut de Myologie
2018-2024
Centre de Recherche en Myologie
2018-2024
Genethon (France)
2010-2017
Mayo Clinic
2014-2017
Mayo Clinic in Arizona
2016
Ruhr University Bochum
2016
WinnMed
2015
Centre National de la Recherche Scientifique
2006-2008
Clathrin-mediated endocytosis has characteristic features in neuronal dendrites and presynapses, but how membrane proteins are internalized along the axon shaft remains unclear. We focused on clathrin-coated structures initial segment (AIS) their relationship to periodic actin-spectrin scaffold that lines axonal plasma membrane. A combination of super-resolution microscopy platinum-replica electron cultured neurons revealed AIS pits form within “clearings”, circular areas devoid mesh....
Background— Left atrial (LA) compliance and contractility influence left ventricular stroke volume. We hypothesized that diminished LA contractile function occur early during the development of heart failure with preserved ejection fraction (HFpEF) impair overall cardiac performance. Methods Results— Cardiac magnetic resonance imaging, echocardiography, pressure-volume studies, tissue analyses were performed in a model HFpEF (elderly dogs, renal wrap-induced hypertension, exogenous...
Excitation-contraction coupling requires a highly specialized membrane structure, the triad, composed of plasma invagination, T-tubule, surrounded by two sarcoplasmic reticulum terminal cisternae. Although precise mechanisms governing T-tubule biogenesis and triad formation remain largely unknown, studies have shown that caveolae participate in mutations several their constituents induce muscle weakness myopathies. Here, we demonstrate that, at membrane, Bin1 caveolin-3 assemble into...
Designed histidine-rich amphipathic cationic peptides, such as LAH4, have enhanced membrane disruption and antibiotic properties when the peptide adopts an alignment parallel to surface. Although this was previously achieved by lowering pH, here we designed a new generation of peptides that adopt surface at neutral pH. In vitro, are powerful antibiotics in terms concentrations required for activity; spectrum target bacteria, fungi, parasites; speed with which they kill. Further modifications...
Breast cancer radiotherapy increases the risk of heart failure with preserved ejection fraction (HFpEF). Cardiomyocytes are highly radioresistant, but radiation specifically affects coronary microvascular endothelial cells, subsequent inflammation and rarefaction. The effects on left ventricular (LV) diastolic function poorly characterized. We hypothesized that cardiac exposure may result in dysfunction without reduced EF. Global expression sodium-iodide symporter (NIS) was induced by...
The histidine‐rich designer peptides of the LAH4 family exhibit potent antimicrobial, transfection, transduction and cell‐penetrating properties. They form non‐covalent complexes with their cargo, which often carry a negative overall charge at pH 7.4 include large range molecules structures such as oligonucleotides, including siRNA DNA, peptides, proteins, nanodots adeno‐associated viruses. These are thought to enter cells through an endosomal pathway where acidification organelle is...
Clathrin plaques are stable features of the plasma membrane observed in several cell types. They abundant muscle, where they localize at costameres that link contractile apparatus to sarcolemma and connect basal lamina. Here, we show clathrin surrounding branched actin filaments form microdomains anchor a three-dimensional desmin intermediate filament (IF) web. Depletion plaque components causes accumulation tangles cytoplasm. We dynamin 2, whose mutations cause centronuclear myopathy (CNM),...
Clathrin function directly derives from its coat structure, and while endocytosis is mediated by clathrin-coated pits, large plaques contribute to cell adhesion. Here, we show that the alternative splicing of a single exon clathrin heavy chain gene (CLTC 31) helps determine organization. Direct genetic control was demonstrated forced CLTC 31 skipping in muscle cells reverses plasma membrane content pits promoting inclusion stimulated flat plaque assembly. Interestingly, mis-splicing found...
Chrysophsin-1 is an amphipathic α-helical antimicrobial peptide produced in the gill cells of red sea bream. The has broad range activity against both Gram-positive and Gram-negative bacteria but more hemolytic than other peptides such as magainin. Here we explore membrane interaction chrysophsin-1 determine its toxicity, vitro, for human lung fibroblasts to obtain a mechanism understand role unusual C-terminal RRRH sequence. At intermediate concentrations, solid-state NMR methods reveal...
Heart failure is a leading cause of morbidity and mortality, cardiac gene delivery has the potential to provide novel therapeutic approaches. Adeno-associated virus serotype 9 (AAV9) transduces rodent heart efficiently, but cardiotropism, immune tolerance, optimal strategies in large animals are unclear. In this study, an AAV9 vector encoding canine sodium iodide symporter (NIS) was administered adult immunocompetent dogs via epicardial injection, coronary infusion without with...
Abstract Neuronal clathrin-mediated endocytosis has unique features in compartments such as dendrites and presynaptic boutons, but how membrane extracellular components are internalized along the axon shaft remains poorly known. Here we focused on clathrin-coated structures initial segment (AIS), their relationship to periodic actin-spectrin scaffold that lines axonal plasma membrane. Super-resolution optical microscopy, platinum replica electron correlative combination cultured hippocampal...
Background Adeno-associated virus has attracted great attention as vehicle for body-wide gene delivery. However, the successful treatment of a disease such Duchenne muscular dystrophy infusion very large amounts vectors is required. This not only raises questions about technical feasibility scale production but also overall safety approach. One way to overcome these problems would be find strategies able increase in vivo efficiency. Methodology Here, we investigated whether polymers can act...
Successful gene therapy of many genetic diseases requires efficient delivery the to several tissues organism. Adeno-associated virus (AAV) is, date, sole vehicle that allows achieving this result but only at condition administering very large amounts vectors. This, however, raises questions about feasibility large-scale production and safety approach. One way overcome both problems would be develop strategies increase in vivo efficiency. Here, we investigated effect fasting on transduction...
ABSTRACT Myotonic dystrophy type 1 (DM1) is a multisystemic neuromuscular disease characterized by CTG repeat expansion in the 3’ untranslated region of gene coding for dystrophia myotonica protein kinase (DMPK). Presence expanded repeats DMPK-mRNAs leads to sequestration RNA binding factors such as Muscleblind like (MBNL) proteins resulting widespread splicing defects contributing progressive muscle weakness and myotonia. Here, we show that abnormal SORBS1 exon 25 found skeletal myotonic...
Abstract Large flat clathrin plaques are stable features of the plasma membrane associated with sites strong adhesion suggesting that they could also play a role in force transduction. Here, we analyzed how interact cytoskeleton and respond to mechanical cues skeletal muscle myotubes. We show branched actin networks surrounding directly regulated by dynamin 2, anchor intermediate filaments sequester YAP at membrane. Dynamin desmin all required for basal nucleocytoplasmic distribution...
Background: Reduced left atrial (LA) compliance (LAC) contributes to pulmonary venous hypertension in heart failure with preserved ejection fraction (HFpEF); however the underlying mechanisms are poorly defined. We hypothesized that microvascular inflammation rarefaction and impaired nitric oxide (NO) signaling, LA titin hypophosphorylation fibrosis may each contribute reduced LAC HFpEF. Methods: Invasive pressure-volume (PV) analyses (open-chest; pericardiotomy; admittance catheter) were...
<h3>Background</h3> Left atrial (LA) compliance and contractility are key determinants of left ventricular (LV) stroke volume. LA remodelling dysfunction in heart failure with preserved ejection fraction (HFpEF) considered to be late presenting features, secondary hypertension-induced LV diastolic chronic pressure overload. However, a recent unifying hypothesis has stipulated that systemic coronary microvascular inflammation drive HFpEF pathophysiology. Considering this paradigm, we...