A5030471987- Autoimmune Bullous Skin Diseases
- Wnt/β-catenin signaling in development and cancer
- Skin and Cellular Biology Research
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Hair Growth and Disorders
- Urticaria and Related Conditions
- Dermatology and Skin Diseases
- Allergic Rhinitis and Sensitization
- Platelet Disorders and Treatments
- Cell Adhesion Molecules Research
- Cancer-related gene regulation
- Food Allergy and Anaphylaxis Research
- Acute Lymphoblastic Leukemia research
- Virus-based gene therapy research
- melanin and skin pigmentation
- Nail Diseases and Treatments
- Wound Healing and Treatments
- Biochemical and Molecular Research
- Hedgehog Signaling Pathway Studies
- HIV/AIDS drug development and treatment
- Cardiovascular Effects of Exercise
- Infectious Diseases and Mycology
- Dermatological and Skeletal Disorders
- Genetics and Physical Performance
- Immune Response and Inflammation
University of Bern
2014-2024
University Hospital of Bern
2014-2024
Karolinska Institutet
2022
Molecular Partners (Switzerland)
2021
University of Lübeck
2006
Northwestern University
2005
University of Amsterdam
1998-2000
Emma Kinderziekenhuis
2000
Johns Hopkins University
1999
Spaarne Ziekenhuis
1997
In pemphigus vulgaris (PV), autoantibody binding to desmoglein (Dsg) 3 induces loss of intercellular adhesion in skin and mucous membranes. Two hypotheses are currently favored explain the underlying molecular mechanisms: (a) disruption through steric hindrance, (b) interference desmosomal cadherin-bound antibody with intracellular events, which we speculated involve plakoglobin. To investigate second hypothesis established keratinocyte cultures from plakoglobin knockout (PG−/−) embryos...
Mutations in the plakoglobin (JUP) gene have been identified arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. However, mechanisms underlying dysfunction involved pathogenesis of ARVC remain poorly understood. Plakoglobin is a component both desmosomes and adherens junctions located at intercalated disc (ICD) cardiomyocytes, where it functions to link cadherins cytoskeleton. In addition, as signaling protein via its ability modulate Wnt/β-catenin pathway. To investigate role...
Plakoglobin (PG) is a member of the Armadillo family adhesion/signaling proteins and has been shown to play critical role in organization desmosomes tissue integrity. Because dissolution intercellular junctions frequently an initial step onset epithelial cell migration, we examined whether loss PG promotes motility by compromising adhesive strength. Keratinocyte cultures established from PG–/–mice exhibited weakened adhesion increased transwell migration assays; both were restored...
Pemphigus vulgaris (PV) is fascinating to dermatologists, epithelial biologists and immunologists alike, as its pathogenesis has been clarified a much greater extent than that of most other organ-specific autoimmune diseases, it provided abundant novel insights into desmoglein biology pathology along the way. Historically, influential PV concept Stanley Amagai. This holds autoantibodies against desmogleins are both essential sufficient for epidermal blister formation (acantholysis) by...
Plakoglobin (PG) is a member of the Armadillo family adhesion/signaling proteins that can be incorporated into both adherens junctions and desmosomes. Loss PG results in defects mechanical integrity heart skin decreased adhesive strength keratinocyte cultures established from knock-out (PG-/-) mice, latter which cannot compensated for by overexpressing closely related β-catenin. In this study, we examined mechanisms PG-regulated adhesion murine keratinocytes. Biochemical morphological...
Desmosome diseases are caused by dysfunction of desmosomes, which anchor intermediate filaments (IFs) at sites cell-cell adhesion. For many decades, the focus attention has been on role actin filament-associated adherens junctions in development and disease, especially cancer. However, interference with function their molecular constituents or attachments to IFs now emerged as a major contributor variety affecting different tissues organs including skin, heart digestive tract. The first...
BackgroundMeprin (EC 3.4.24.18), an astacin-like metalloprotease, is expressed in the epithelium of intestine and kidney tubules has been related to cancer, but mechanistic links are unknown.Methodology/Principal FindingsWe used MDCK Caco-2 cells stably transfected with meprinα or meprinβ establish models renal intestinal epithelial expressing this protease at physiological levels. In both E-cadherin was cleaved, producing a cell-associated 97-kDa fragment, which enhanced upon activation...
Evidence has accumulated that changes in intracellular signaling downstream of desmoglein 3 (Dsg3) may have a significant role epithelial blistering the autoimmune disease pemphigus vulgaris (PV). Currently, most studies on PV involve passive transfer pathogenic antibodies into neonatal mice not finalized epidermal morphogenesis, and do permit analysis mature hair follicles (HFs) stem cell niches. To investigate Dsg3 antibody-induced adult epidermis at defined stages HF cycle, we developed...
Hereditary footpad hyperkeratosis (HFH) represents a palmoplantar hyperkeratosis, which is inherited as monogenic autosomal recessive trait in several dog breeds, such e.g. Kromfohrländer and Irish Terriers. We performed genome-wide association studies (GWAS) both breeds. In we obtained single strong signal on chromosome 5 (praw = 1.0×10−13) using 13 HFH cases 29 controls. The replicated an independent cohort of Terriers with 10 21 controls 6.9×10−10). analysis shared haplotypes among the...
Sequence analysis of the adhesion molecule E-cadherin had revealed a multibasic motif [4PArg-Gln-Lys-Arg1P], reminiscent minimal cleavage signal for furin, prototype proprotein convertase family, and/or other members sharing similar sequence specificity. Mutation this site was sufficient to abolish processing in fibroblasts reinforcing possibility that convertases are involved maturation molecule. Here we demonstrate even though furin can efficiently and specifically cleave proE-cadherin...
Ichthyoses are a heterogeneous group of inherited cornification disorders characterized by generalized dry skin, scaling and/or hyperkeratosis. Ichthyosis vulgaris is the most common form ichthyosis in humans and caused genetic variants FLG gene encoding filaggrin. Filaggrin key player formation stratum corneum, uppermost layer epidermis therefore crucial for barrier function. During terminal differentiation keratinocytes, precursor profilaggrin cleaved several proteases into filaggrin...
Polyurethane-based hydrogels are relatively inexpensive and mechanically robust biomaterials with ideal properties for various applications, including drug delivery, prosthetics, implant coatings, soft robotics, tissue engineering. In this report, a simple method is presented synthesizing casting biocompatible polyurethane-poly(ethylene glycol) (PU-PEG) tunable mechanical properties, nonfouling characteristics, sustained tolerability as an implantable material or coating. The synthesized via...
The selection of ECG leads used for ST monitoring may influence detection and quantitation ischaemia.We compared on-line continuous 48-h 12-lead against 3-lead in 130 unstable angina patients (Mortara. ELI-100). Onset offset episodes were defined by the lead with first > or = 100 microV change relative to baseline latest return level, respectively. calculated 12 3 (V2, V5, III) separately.ST detected 88 (77%) 71 (62%) (P < 0.02). median number (25.75%) episodes/patient was 1 (0.3) 2 (1.6)...
Hereditary nasal parakeratosis (HNPK), an inherited monogenic autosomal recessive skin disorder, leads to crusts and fissures on the planum of Labrador Retrievers. We performed a genome-wide association study (GWAS) using 13 HNPK cases 23 controls. obtained single strong signal chromosome 2 (praw = 4.4×10−14). The analysis shared haplotypes among defined critical interval 1.6 Mb with 25 predicted genes. re-sequenced genome one case at 38× coverage detected 3 non-synonymous variants in...