A5014968495- Cell Adhesion Molecules Research
- Osteoarthritis Treatment and Mechanisms
- Proteoglycans and glycosaminoglycans research
- Muscle Physiology and Disorders
- Protease and Inhibitor Mechanisms
- Connective tissue disorders research
- Cellular Mechanics and Interactions
- RNA Research and Splicing
- Glycosylation and Glycoproteins Research
- Collagen: Extraction and Characterization
- Ocular Disorders and Treatments
- Immune Response and Inflammation
- Wound Healing and Treatments
- Platelet Disorders and Treatments
- Bone and Dental Protein Studies
- Bacillus and Francisella bacterial research
- Skin and Cellular Biology Research
- Peptidase Inhibition and Analysis
- Congenital heart defects research
- Corneal Surgery and Treatments
- Zebrafish Biomedical Research Applications
- Nerve injury and regeneration
- Cardiomyopathy and Myosin Studies
- Cancer-related molecular mechanisms research
- TGF-β signaling in diseases
University of Cologne
2015-2024
North-West University
2024
University Hospital Cologne
2019-2022
Center for Molecular Medicine and Immunology
2016
Institute of Molecular Biology and Biophysics
2014
Cologne Excellence Cluster on Cellular Stress Responses in Aging Associated Diseases
2011-2014
Friedrich-Alexander-Universität Erlangen-Nürnberg
2002
Leco Corporation (United States)
1997
Native supramolecular assemblies containing collagen VI microfibrils and associated extracellular matrix proteins were isolated from Swarm rat chondrosarcoma tissue. Their composition spatial organization characterized by electron microscopy immunological detection of molecular constituents. The small leucine-rich repeat (LRR) proteoglycans biglycan decorin bound to the N-terminal region VI. Chondroadherin, another member LRR family, was identified both at N C termini Matrilin-1, -3, -4...
Here we describe a novel collagen belonging to the class of von Willebrand factor A (VWA) domain-containing proteins. This protein was identified by screening EST data base and subsequently recombinantly expressed characterized as an authentic tissue component. The COL28A1 gene on human chromosome 7p21.3 mouse 6A1 encodes that structurally resembles beaded filament-forming collagens. collagenous domain contains several very short interruptions arranged in repeat pattern. As shown for other...
The matrilins are a family of four noncollagenous oligomeric extracellular matrix proteins with modular structure. Matrilins can act as adapters which bridge different macromolecular networks. We therefore investigated the effect collagen IX deficiency on matrilin-3 integration into cartilage tissues. Mice harboring deleted Col9a1 gene lack synthesis functional protein and produce fibrils completely devoid IX. Newborn knockout mice exhibited significantly decreased (COMP) signals,...
Using forward genetics, we have identified the genes mutated in two classes of zebrafish fin mutants. The mutants first class are characterized by defects embryonic morphogenesis, which due to mutations a Laminin subunit or an Integrin alpha receptor, respectively. second display characteristic blistering underneath basement membrane epidermis. Three them orthologues FRAS1, FREM1, FREM2, large protein encoding that mouse bleb and human patients suffering from Fraser Syndrome, rare congenital...
Classical osteogenesis imperfecta (OI) is a bone disease caused by type I collagen mutations and characterized fragility, frequent fractures in absence of trauma growth deficiency. No definitive cure available for OI to develop novel drug therapies, taking advantage repositioning strategy, the small teleost zebrafish (Danio rerio) particularly appealing model. Its size, high proliferative rate, embryo transparency amount required make model choice screening studies, when valid available. We...
The extracellular matrix (ECM) provides structural support for tissue architecture and is a major effector of cell behavior during skin repair inflammation. Macrophages are involved in all stages but only limited knowledge exists about macrophage-specific expression regulation ECM components. In this study, we used transcriptome profiling bioinformatic analysis to define the unique ECM-associated genes cultured macrophages. Characterization matrisome revealed that most were constitutively...
Matrilin-3 is a recently identified member of the superfamily proteins containing von Willebrand factor A-like domains and able to form hetero-oligomers with matrilin-1 (cartilage matrix protein) via C-terminal coiled-coil domain. Full-length matrilin-3 fragment lacking assembly domain were expressed in 293-EBNA cells, purified, subjected biochemical characterization. Recombinantly full-length occurs as monomers, dimers, trimers, tetramers, detected by electron microscopy SDS-polyacrylamide...
The clinical application of advanced next-generation sequencing technologies is increasingly uncovering novel classes mutations that may serve as potential targets for precision medicine therapeutics. Here, we show a deep intronic splice defect in the COL6A1 gene, originally discovered by applying muscle RNA patients with findings collagen VI–related dystrophy (COL6-RD), inserts an in-frame pseudoexon into mRNA, encodes mutant α1(VI) protein exerts dominant-negative effect on VI matrix...
In response to injury, tendon fibroblasts are activated, migrate the wound, and contribute tissue repair by producing organizing extracellular matrix. Collagen VI is a microfibrillar collagen enriched in pericellular matrix of with potential regulatory role mechanism. We investigated molecular basis interaction between cell membrane both sections fibroblast cultures human tendon, analyzed deposition during migration myofibroblast trans-differentiation, two crucial events for repair. Tendon...
Snake venom metalloproteinases (SVMPs) are members of the Reprolysin family to which ADAM (a disintegrin and metalloproteinase) proteins also belong. The disintegrin-like/cysteine-rich domains ADAMs have been implicated in their function. In case SVMPs, we hypothesized that these could function target key extracellular matrix or cell surface proteins. Initially detected interaction collagen XIV, a fibril-associated with interrupted triple helices containing von Willebrand factor A (VWA)...
A mouse cDNA encoding for matrilin‐3, the third member of novel matrilin family extracellular matrix proteins, was cloned. The protein precursor 481 amino acids consists a putative signal peptide, short positively charged sequence, single vWFA‐like domain followed by four epidermal growth factor‐like modules and potential coiled‐coil α‐helical oligomerization at C‐terminus. It is smallest with predicted M r mature 48 902. primary structure C‐terminal portion 310 human matrilin‐3 determined...
Disruption to endochondral ossification leads delayed and irregular bone formation can result in a heterogeneous group of genetic disorders known as the chondrodysplasias. One such disorder, multiple epiphyseal dysplasia (MED), is characterized by mild dwarfism early-onset osteoarthritis from mutations gene encoding matrilin-3 ( MATN3 ). To determine disease mechanisms that underpin pathophysiology MED we generated murine model knocking-in matn3 mutation. Mice are homozygous for mutation...
Matrilins are oligomeric extracellular matrix adaptor proteins mediating interactions between collagen fibrils and other constituents. All four matrilins expressed in cartilage mutations the human gene encoding matrilin-3 (MATN3) associated with different forms of chondrodysplasia. Surprisingly, however, Matn3-null as well Matn1- Matn2-null mice do not show an overt skeletal phenotype, suggesting a dominant negative pathomechanism for disorders redundancy/compensation among family members...