A5049241669- Muscle Physiology and Disorders
- Skin and Cellular Biology Research
- Cellular Mechanics and Interactions
- Genetic Neurodegenerative Diseases
- Cardiomyopathy and Myosin Studies
- RNA and protein synthesis mechanisms
- Bacterial Genetics and Biotechnology
- Microtubule and mitosis dynamics
- Nuclear Structure and Function
- Autophagy in Disease and Therapy
- RNA modifications and cancer
- Enzyme Structure and Function
- Silk-based biomaterials and applications
- Elasticity and Material Modeling
- 3D Printing in Biomedical Research
- Inflammatory Myopathies and Dermatomyositis
- Endoplasmic Reticulum Stress and Disease
- Heavy Metal Exposure and Toxicity
- Tissue Engineering and Regenerative Medicine
- Lysosomal Storage Disorders Research
- Genomics and Phylogenetic Studies
- Toxoplasma gondii Research Studies
- Genomics and Chromatin Dynamics
- Sphingolipid Metabolism and Signaling
- Amino Acid Enzymes and Metabolism
Université Paris Cité
2012-2023
Centre National de la Recherche Scientifique
2003-2023
Unit of Functional and Adaptive Biology
2010-2022
Physiologie et Médecine Expérimentale du Coeur et des Muscles
2018
Sorbonne Paris Cité
2013-2017
Sorbonne Université
2017
Délégation Paris 7
2012
École Polytechnique
1992-2003
Disruption of mitochondrial function and protein homeostasis plays a central role in aging. However, how these processes interact what governs their failure aging remain poorly understood. Here, we showed that ceramide biosynthesis controls the decline during muscle Analysis transcriptome datasets derived from biopsies obtained both aged individuals patients with diverse range disorders revealed changes biosynthesis, as well disturbances pathways, are prevalent features conditions. By...
In Saccharomyces cerevisiae , disruption of the YCF1 gene increases sensitivity cell growth to mercury. Transformation resulting ycf1 null mutant with a plasmid harbouring under control GAL promoter largely restores wild‐type resistance metal ion. The protective effect Ycf1p against toxicity mercury is especially pronounced when yeast cells are grown in rich medium or minimal supplemented glutathione. Secretory vesicles from S. overproducing shown exhibit ATP‐dependent transport...
The yihZ gene of Escherichia coli is shown to produce a deacylase activity capable recycling misaminoacylated d-Tyr-tRNATyr. reaction specific and, under optimal in vitroconditions, proceeds at rate 6 s−1 with aK m value for the substrate equal 1 μm. Cell growth sensitive interruption theyihZ if d-tyrosine added minimal culture medium. Toxicity exogenous exacerbated if, addition disruption yihZ, d-amino acid dehydrogenase (dadA) also inactivated. Orthologs occur many, but not all, bacteria....
The mechanical properties of cells are essential to maintain their proper functions, and mainly rely on cytoskeleton. A lot attention has been paid actin filaments, demonstrating central role in the properties, but much less is known about participation intermediate filament (IF) networks. Indeed contribution IFs, such as vimentin, keratins lamins, cell mechanics only assessed recently. We study here involvement desmin, an IF specifically expressed muscle cells, rheology immature cells....
Desminopathies, a subgroup of myofibrillar myopathies (MFMs), the progressive muscular diseases characterized by accumulation granulofilamentous desmin-positive aggregates, result from mutations in desmin gene (DES), encoding muscle-specific intermediate filament. Desminopathies often lead to severe disability and premature death cardiac and/or respiratory failure; no specific treatment is currently available. To identify drug-targetable pathophysiological pathways, we performed...
Mutations within the human desmin gene are responsible for a subcategory of myofibrillar myopathies called desminopathies. However, single inherited mutation can produce different phenotypes family, suggesting that environmental factors influence disease states. Although several mouse models have been used to investigate organ-specific desminopathies, more general mechanistic perspective is required advance our knowledge toward patient treatment. To improve understanding pathology, we...
The ubiquitin proteasome system and macroautophagy are proteolytic pathways essential in the maintenance of cellular homeostasis during differentiation remodelling skeletal muscle. In both pathways, proteins to be degraded tagged with polyubiquitin. muscles, MURF2 display E3 ligase structure suggesting that they may covalently attach polypeptides still unknown target proteins. So far only MURF2A isoforms were studied shown interact p62/SQSTM1, a protein implicated macroautophagic...
Cellular adhesion and migration are key functions that disrupted in numerous diseases. We report desmin, a type-III muscle-specific intermediate filament, is novel cell regulator. Expression of p.R406W mutant identified patients with desmin-related myopathy, modified focal area expression adhesion-signaling genes myogenic C2C12 cells. Satellite cells extracted from desmin-knock-out (DesKO) desmin-knock-in-p.R405W (DesKI-R405W) mice were less adhesive migrated faster than those wild-type...
Mechanics is now recognized as crucial in cell function. To date, the mechanical properties of cells have been inferred from experiments which investigate roles actin and microtubules ignoring intermediate filaments (IFs) contribution. Here, we analyse myoblasts behaviour context myofibrillar myopathy resulting p.D399Y desmin mutation disorganizes IF network muscle cells. We compare response expressing either mutated or wild-type to cyclic stretch. Cells are cultivated on supports submitted...
Liquid and elastic behaviours of tissues drive their morphology response to the environment. They appear as first insight into tissue mechanics. We explore role individual cell properties on spheroids mouse muscle precursor cells investigate intermediate filaments surface tension Young’s modulus. By flattening multicellular myoblast aggregates under magnetic constraint, we measure rigidity show that they act highly sensitive macroscopic reporters closely related microscopic local effective...
Using random Tn 10 insertion mutagenesis, we isolated an Escherichia coli mutant strain affected in the regulation of lysU , gene encoding inducible form lysyl‐tRNA synthetase. The transposon giving rise to altered expression was found inserted within lrp . latter codes for leucine‐responsive regulatory protein (Lrp) which mediates a global response bacterium leucine. An involvement Lrp searched by using lysU‐lacZ operon fusion. following conclusions were reached: (i) inactivation causes...
Background: Mutations in the desmin gene cause skeletal myopathies and cardiomyopathies. The objective of this study was to elucidate molecular pathology induced by expression R405W mutant murine muscle tissue. Methods: A comprehensive characterization hetero- homozygous knock-in mice performed, employing grip strength, blood acylcarnitine amino acid, histological, ultrastructural, immunofluorescence, immunoblot, ribosomal stalling, RNA sequencing proteomic analyses. Results: Both showed...
Biological tissues achieve proper shape and ordered structures during development through responses to internal external signals, with mechanical cues playing a crucial role. These forces guide cellular organization, leading complex self-organizing that are foundational embryonic patterns. Emerging theories experiments suggest “topological morphogens” drive these processes. Despite the predominance of three-dimensional (3D) in biology, studying 3D remains challenging due limited model...
Abstract Liquid and elastic behavior of tissues drives their morphology response to the environment. They appear as first insight on tissue mechanics. We explore role individual cell properties spheroids mouse muscle precursor cells by developing a fully automated surface tension Young’s modulus measurement system. Flattening multicellular aggregates under magnetic constraint, we show that rigidity act highly sensitive macroscopic reporters closely related microscopic local effective...