A5030731723- Mitochondrial Function and Pathology
- Ubiquitin and proteasome pathways
- Hereditary Neurological Disorders
- Autophagy in Disease and Therapy
- interferon and immune responses
- Neurological diseases and metabolism
- Genetic Neurodegenerative Diseases
- Adipose Tissue and Metabolism
- NF-κB Signaling Pathways
- Sirtuins and Resveratrol in Medicine
- Cholesterol and Lipid Metabolism
- Endoplasmic Reticulum Stress and Disease
- Virus-based gene therapy research
- Click Chemistry and Applications
- Cardiovascular Function and Risk Factors
- Atherosclerosis and Cardiovascular Diseases
- Genetic Syndromes and Imprinting
- Protein Degradation and Inhibitors
- Neurogenetic and Muscular Disorders Research
- RNA modifications and cancer
- Retinal Development and Disorders
- Redox biology and oxidative stress
- Angiogenesis and VEGF in Cancer
- Birth, Development, and Health
- Circular RNAs in diseases
Maladies Rares: Génétique et Métabolisme
2012-2023
Université de Bordeaux
2012-2023
Inserm
1999-2023
Hôpital Pellegrin
2012-2016
Biotherapy of Genetic Diseases, Inflammatory Disorders and Cancers
2013-2015
Neurocentre Magendie
2012
Centre Hospitalier Universitaire de Bordeaux
2012
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...
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...
The hereditary spastic paraplegias are an expanding and heterogeneous group of disorders characterized by spasticity in the lower limbs. Plasma biomarkers needed to guide genetic testing paraplegia. Spastic paraplegia type 5 (SPG5) is autosomal recessive due mutations CYP7B1, which encodes a cytochrome P450 7α-hydroxylase implicated cholesterol bile acids metabolism. We developed method based on ultra-performance liquid chromatography electrospray tandem mass spectrometry validate two plasma...
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...
Objectives. Mitochondrial DNA (mtDNA) contains sequestered damage-associated molecular patterns that might be involved in osteoimmunological pathogenesis of RA. Here, we aimed to investigate the cellular source mtDNA and its role RANK ligand (RANKL) expression by RA SF neutrophils. Methods. The gene signature neutrophils was examined proteomic quantitative analysis. Levels circulating from patients OA control subjects were assessed real-time PCR. Purified challenged vitro with Toll-like...
Cellular energy homeostasy relies on mitochondrial plasticity, the molecular determinants of which are multiple. Yet, relative contribution and possible cooperation between biogenesis morphogenesis to cellular remains elusive. Here we analyzed adaptative capacity content dynamics in muscle biopsies patients with a complex IV defect, skin fibroblasts challenged inhibition.We observed biphasic variation upon inhibition fibroblasts. Adjustment for respiratory maintenance was blocked by using...
Vascular cell adhesion molecule-1 (VCAM-1) gene expression in cytokine-activated cells depends on two κB elements. Since VCAM-1 appears developmentally regulated and cytokine-inducible smooth muscle (SMCs), we have studied the role of NF-κB differentiated SMC expression. Confluent SMCs were cultured either a serum-free medium order to induce differentiation, or with serum, stimulated not by tumor necrosis factor α (TNF-α). The myosin heavy chain, marker, was induced concomitantly medium,...
In mammals, about 99% of mitochondrial proteins are synthesized in the cytosol as precursors that subsequently imported into organelle. The health and functions rely on an accurate quality control these proteins. Here, we show E3 ubiquitin ligase F box/leucine-rich-repeat protein 6 (FBXL6) regulates cytosolically translated Indeed, found FBXL6 substrates newly ribosomal This binds to chaperones involved folding trafficking peptide ribosomal-associated Deletion interacting partners is...
Dufourcq, Pascale; Louis, Huguette; Dandre, Frederic; Lavie, Julie; Bonnet, Jacques; Lamaziere, Jean-Marie Daniel Author Information
Because tissue freeze-drying is an excellent way to preserve antigenic conformation, we have tested the feasibility of this technique reveal nonradioactive in situ hybridization (ISH) mRNA. We compared mRNA detection after different methods preservation, freeze-drying, cryosectioning, and formaldehyde or methanol fixation. Our results show that ISH more sensitive for tissues preserved by than other preparations. demonstrated allows combination immunohistochemistry simultaneous antigen...
Summary The large majority of mitochondrial proteins is synthesized in the cytosol and then imported to organelle. To ensure proper functions, quality these needs be guaranteed. Here, we show that E3 ubiquitin ligase F-box/LRR-repeat protein 6 (FBXL6) participates at level cytosolic translation. We found lack FBXL6 has severe effects including ribosomal aggregations, altered metabolism inhibited cell cycle progression oxidative conditions. was interact specifically with ribosomal-associated...