A5004078749- Mitochondrial Function and Pathology
- Cancer, Hypoxia, and Metabolism
- ATP Synthase and ATPases Research
- Metabolism and Genetic Disorders
- Metabolomics and Mass Spectrometry Studies
- Adipose Tissue and Metabolism
- RNA modifications and cancer
- Protein Tyrosine Phosphatases
- Genetic Neurodegenerative Diseases
- Peptidase Inhibition and Analysis
- Bioinformatics and Genomic Networks
- Autophagy in Disease and Therapy
- Mercury impact and mitigation studies
- Heart Rate Variability and Autonomic Control
- Nitric Oxide and Endothelin Effects
- Cancer, Lipids, and Metabolism
- Ubiquitin and proteasome pathways
- Pulmonary Hypertension Research and Treatments
- Aquaculture Nutrition and Growth
- Coenzyme Q10 studies and effects
- Protein Degradation and Inhibitors
- Carbohydrate Chemistry and Synthesis
- Receptor Mechanisms and Signaling
- Pain Management and Opioid Use
- Histone Deacetylase Inhibitors Research
Inserm
2008-2023
Université de Bordeaux
2011-2023
Bordeaux Population Health
2007-2022
Maladies Rares: Génétique et Métabolisme
2010-2020
Hôpital Pellegrin
2016
Rice University
2010-2014
Center for Systems Biology
2012
Centre Hospitalier Universitaire de Bordeaux
2011
Biotherapy of Genetic Diseases, Inflammatory Disorders and Cancers
2010
Laboratoire de Biogenèse Membranaire
2008
Mitochondria form a dynamic network, and it remains unclear how the alternate configurations interact with bioenergetics properties. The metabolic signals that link mitochondrial structure to its functional states have not been fully characterized. In this report, we analyze bidirectional relationships between morphology function in living human cells. First, determined effect of fission on energy production by using small interfering RNA (siRNA) targeting DRP1, which revealed importance...
To investigate the physiological diversity in regulation and control of mitochondrial oxidative phosphorylation, we determined composition functional features respiratory chain muscle, heart, liver, kidney, brain. First, observed important variations content infrastructure via electron micrographs different tissue sections. Analyses enzyme by Western blot also showed large differences between tissues, good correlation with expression level transcription factor A activity citrate synthase. On...
Podocytes play a key role in diabetic nephropathy pathogenesis, but alteration of their metabolism remains unknown human kidney. By using conditionally differentiating podocyte cell line, we addressed the functional and molecular changes energetics during vitro development or under high glucose conditions. In 5 mM medium, observed stepwise activation oxidative differentiation that was characterized by peroxisome proliferator-activated receptor-γ coactivator 1α (PGC-1α)-dependent stimulation...
Anoikis resistance, or the ability for cells to live detached from extracellular matrix, is a property of epithelial cancers. The “Warburg effect,” preference cancer glycolysis their energy production even in presence oxygen, has been shown be evident various tumors. Since cell's metastatic depends on microenvironmental conditions (nutrients, stromal cells, and vascularization) highly variable different organs, cellular metabolic fluxes nutrient demand may show considerable differences....
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by mutations in the SACS gene. encodes sacsin, a protein whose function remains unknown, despite description numerous domains and recent focus on its potential role regulation mitochondrial physiology. This study aimed to identify new large population ataxic patients functionally analyze their cellular effects compartment. A total 321 index with selected from SPATAX network were analyzed direct sequencing gene, 156...
DNA damage is a well-known initiator of tumorigenesis. Studies have shown that most cancer cells rely on aerobic glycolysis for their bioenergetics. We sought to identify molecular link between genomic mutations and metabolic alterations in neoplastic transformation. took advantage the intrinsic instability arising xeroderma pigmentosum C (XPC). The XPC protein plays key role recognizing nucleotide excision repair, patients with deficiency increased incidence skin other malignancies. In...
Metabolic reprogramming is a common hallmark of cancer, but large variability in tumor bioenergetics exists between patients. Using high-resolution respirometry on fresh biopsies human lung adenocarcinoma, we identified 2 subgroups reflected the histologically normal, paired, cancer-adjacent tissue: high (OX+) mitochondrial respiration and low (OX-) respiration. The OX+ tumors poorly incorporated [18F]fluorodeoxy-glucose showed increased expression trifunctional fatty acid oxidation enzyme...
Mammalian ATAD3 is a mitochondrial protein, which thought to play an important role in nucleoid organization. However, its exact function still unresolved.Here, we characterize the Caenorhabditis elegans (C. elegans) homologue (ATAD-3) and investigate importance for development. We show that ATAD-3 highly conserved among different species RNA mediated interference against atad-3 causes severe defects, characterized by early larval arrest, gonadal dysfunction embryonic lethality....
Germline mutations that activate genes in the canonical RAS/MAPK signaling pathway are responsible for rare human developmental disorders known as RASopathies. Here, we analyzed molecular determinants of Costello syndrome (CS) using a mouse model expressing HRAS p.G12S, patient skin fibroblasts, hiPSC-derived cardiomyocytes, p.G12V zebrafish model, and fibroblasts lentiviral constructs carrying p.G12S or p.G12A mutations. The findings revealed alteration mitochondrial proteostasis defective...
β-adrenoceptor (β-AR)-mediated relaxation was characterized in pulmonary arteries from normoxic and hypoxic (as model of hypertension) mice. The endothelial NO synthase (eNOS) pathway especially investigated because its potential vasculoprotective effects. Pulmonary control or (0.5 atm for 21 days) wild-type eNOS−/− mice were used pharmacological characterization β-AR-mediated myograph, immunohistochemistry using anti-β-AR antibodies. In mice, isoproterenol (β-AR agonist) procaterol...
The histidine triad nucleotide-binding (HINT2) protein is a mitochondrial adenosine phosphoramidase expressed in the liver and pancreas. Its physiological function unknown. To elucidate role of HINT2 physiology, mouse Hint2 gene was deleted. −/− +/+ mice were generated mixed C57Bl6/J × 129Sv background. At 20 weeks, phenotypic changes relative to an accumulation hepatic triglycerides, decreased tolerance glucose, defective counter-regulatory response insulin-provoked hypoglycemia, increase...
Hereditary spastic paraplegia, SPG31, is a rare neurological disorder caused by mutations in REEP1 gene encoding the microtubule-interacting protein, REEP1. The mechanism which REEP1-dependent processes are linked with disease unclear. regulates morphology and trafficking of various organelles via interaction microtubules. In this study, we collected primary fibroblasts from SPG31 patients to investigate their mitochondrial morphology. We observed that patient cells was highly tubular...
Background Local anesthetics offer the benefits of extended analgesia with greater patient satisfaction and faster rehabilitation compared intravenous morphine. These benefits, however, can be offset by adverse iatrogenic muscle pain caused bupivacaine. Here, authors describe mechanisms local anesthetic-induced myotoxicity a partial protective effect recombinant human erythropoietin (rhEPO). Methods The developed rat model femoral nerve catheter cell culture skeletal myoblasts to study...