A5049773464- Skin and Cellular Biology Research
- Cell Adhesion Molecules Research
- Autoimmune Bullous Skin Diseases
- Receptor Mechanisms and Signaling
- Cellular Mechanics and Interactions
- Mass Spectrometry Techniques and Applications
- RNA Interference and Gene Delivery
- Neuropeptides and Animal Physiology
- Dermatological and Skeletal Disorders
- Ion channel regulation and function
- Monoclonal and Polyclonal Antibodies Research
- Viral Infectious Diseases and Gene Expression in Insects
- Protease and Inhibitor Mechanisms
- Immunotherapy and Immune Responses
- Ubiquitin and proteasome pathways
- Peptidase Inhibition and Analysis
- Advanced Proteomics Techniques and Applications
- Hair Growth and Disorders
- Cellular transport and secretion
- Endoplasmic Reticulum Stress and Disease
- Cancer Research and Treatments
- Immune Cell Function and Interaction
- Heat shock proteins research
- Muscle metabolism and nutrition
- Pharmacological Effects and Assays
University of Freiburg
2016-2024
University Medical Center Freiburg
2016-2024
Université de Strasbourg
2002-2023
Centre National de la Recherche Scientifique
2002-2023
Biotechnologie et Signalisation Cellulaire
2013-2023
École Supérieure de Biotechnologie de Strasbourg
2017
Abstract Most membrane proteins studies require the use of detergents, but because lack a general, accurate and rapid method to quantify them, many uncertainties remain that hamper proper functional structural data analyses. To solve this problem, we propose based on matrix-assisted laser desorption/ionization mass spectrometry (MALDI-TOF MS) allows quantification pure or mixed detergents in complex with proteins. We validated wide variety automated process, thereby allowing routine for...
The "generalized severe" form of recessive dystrophic epidermolysis bullosa (RDEB-gen sev) is caused by bi-allelic null mutations in COL7A1, encoding type VII collagen. absence collagen leads to blistering the skin and mucous membranes upon slightest trauma. Because most patients carry exonic point or small insertions/deletions, exons COL7A1 are in-frame, low levels already drastically improve disease phenotype, this gene seems a perfect candidate for antisense oligonucleotide (AON)-mediated...
Genetic loss of collagen VII causes recessive dystrophic epidermolysis bullosa (RDEB), a skin fragility disorder that, unexpectedly, manifests also with elevated colonization commensal bacteria and frequent wound infections. Here, we describe an unprecedented systemic function as member unique innate immune-supporting multiprotein complex in spleen lymph nodes. In this complex, specifically binds sequesters the immune activator cochlin lumen lymphoid conduits. genetic mouse models, increased...
Dystrophic epidermolysis bullosa (DEB) is a blistering skin disease caused by mutations in the gene COL7A1 encoding collagen VII. DEB can be inherited as recessive (RDEB) or dominant (DDEB) and associated with high wound burden. Perpetual cycles of wounding healing drive fibrosis DDEB RDEB, well formation tumor-permissive microenvironment. Prolonging wound-free episodes improving quality would therefore confer substantial benefit for individuals DEB. The collagenous domain VII encoded 82...
Fibronectin serves as a platform to guide and facilitate deposition of collagen fibrillin microfibrils. During development fibrotic diseases, altered fibronectin in the extracellular matrix (ECM) is generally an early event. After this, dysregulated organization fibrillins fibrillar collagens occurs. Because essential orchestrator healthy ECM, perturbation its ECM-organizational capacity may be involved fibrosis. To investigate we employed recessive dystrophic epidermolysis bullosa disease...
Genetically evoked deficiency of collagen VII causes dystrophic epidermolysis bullosa (DEB)—a debilitating disease characterized by chronic skin fragility and progressive fibrosis. Removal exons carrying frame-disrupting mutations can reinstate protein expression in genetic diseases. The therapeutic potential this approach is critically dependent on gene, protein, intrinsic factors. Naturally occurring exon skipping COL7A1, translating VII, suggests that containing disease-causing may be...
The extracellular matrix protein collagen VII is part of the microenvironment stratified epithelia and critical in organismal homeostasis. Mutations encoding gene COL7A1 lead to skin disorder dystrophic epidermolysis bullosa (DEB), are linked fragility progressive inflammation-driven fibrosis that facilitates aggressive cancer. So far, these changes have been mesenchymal alterations, epithelial consequences loss remaining under-addressed. As dysfunction a principal initiator fibrosis, we...
GPCR desensitization and down-regulation are considered key molecular events underlying the development of tolerance in vivo. Among many regulatory proteins that involved these complex processes, GASP-1 have been shown to participate sorting several receptors toward degradation pathway. This protein belongs recently identified GPCR-associated (GASPs) family comprises ten members for which structural functional details poorly documented. We present here a detailed structure–function...
High conservation of extracellular matrix proteins often makes the generation potent species-specific antibodies challenging. For collagen VII there is a particular preclinical interest in ability to discriminate between human and murine VII. Deficiency causes dystrophic epidermolysis bullosa (DEB) – genetic skin blistering disease, which its most severe forms highly debilitating. Advances gene cell therapy approaches have made curative therapies for diseases realistic possibility. DEB one...
Astacin-like proteinases (ALPs) are regulators of tissue and extracellular matrix (ECM) homeostasis. They convey this property through their ability to convert ECM protein pro-forms functional mature proteins by regulating the bioavailability growth factors that stimulate synthesis. The most studied ALPs in context BMP-1/tolloid-like proteinases. other subclass vertebrates – meprins, comprised meprin α β emerging as homeostasis but have so far been only limitedly investigated. Here, we...
Long-term functional stability of isolated membrane proteins is crucial for many in vitro applications used to elucidate molecular mechanisms, and drug screening platforms modern pharmaceutical industry. Compared soluble proteins, the understanding at level remains a challenge. This partly due difficulty isolate simultaneously maintain their structural stability, because hydrophobic nature. Here we show, how scintillation proximity assay can be analyze time-resolved high-affinity ligand...
Abstract Among the small number of expression systems validated for mass production eukaryotic membrane proteins (EMPs), methylotrophic yeast Pichia pastoris stands as one most efficient hosts. This system has been used to produce crystallization‐grade a variety EMPs, from which high‐resolution 3D structures have determined. unit describes set guidelines and instructions overexpress using P. system. Using G protein–coupled receptor (GPCR) model EMP, these protocols illustrate necessary...
<h3>Background</h3> Immunocytokines provide the opportunity to simultaneously address distinct and complementary mechanisms of action deliver cytokine payloads specific immune cells ('cis-signaling') resulting in enhanced anti-tumor activity. However, there is increasing evidence that use constitutively active IL-2 payload limits therapeutic index PD1-IL2 immunocytokines due systemic toxicity from potent induced cell activation release pro-inflammatory cytokines periphery. <h3>Methods</h3>...