Elevated serum levels of sonic hedgehog are associated with fibrotic and vascular manifestations in systemic sclerosis
| Autor: | Serena Vettori, Andreas Ramming, Gabriele Valentini, Jérôme Avouac, Ulf Müller-Ladner, Britta Maurer, Jörg H W Distler, Serena Guiducci, Marco Matucci-Cerinic, Dörte Huscher, Yannick Allanore, Oliver Distler, Veronika K. Jaeger, Florian M P Meier, Georg Schett, Ulrich A Walker, Gabriela Riemekasten, Elise Siegert, Christian Beyer, Christina Bergmann |
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| Přispěvatelé: | Beyer, Christian, Huscher, Dörte, Ramming, Andrea, Bergmann, Christina, Avouac, Jerome, Guiducci, Serena, Meier, Florian, Vettori, Serena, Siegert, Elise, Jaeger, Veronika K, Maurer, Britta, Riemekasten, Gabriela, Walker, Ulrich, Müller Ladner, Ulf, Valentini, Gabriele, Matucci Cerinic, Marco, Allanore, Yannick, Distler, Oliver, Schett, Georg, Distler, Jörg H. W., University of Zurich, Distler, Jörg H W |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Pathology animal structures 2745 Rheumatology Immunology 610 Medicine & health Genetics and Molecular Biology Systemic Sclerosis Autoimmune Disease General Biochemistry Genetics and Molecular Biology Scleroderma Pathogenesis 03 medical and health sciences 0302 clinical medicine Rheumatology Fibrosis 1300 General Biochemistry Genetics and Molecular Biology Internal medicine medicine Immunology and Allergy Sonic hedgehog skin and connective tissue diseases 030203 arthritis & rheumatology 2403 Immunology integumentary system biology business.industry Case-control study 10051 Rheumatology Clinic and Institute of Physical Medicine medicine.disease Phenotype Pulmonary hypertension 030104 developmental biology embryonic structures General Biochemistry biology.protein 2723 Immunology and Allergy Fibroblast business |
| Popis: | Fibrotic tissue remodelling and vascular alterations cause high morbidity and mortality in patients with systemic sclerosis (SSc).1 2 While the pathogenesis of SSc is only partially understood, recent evidence demonstrates a fundamental role of hedgehog signalling in fibrotic disease manifestations. Hedgehog signalling is activated in fibrotic SSc skin, and it induces an SSc-like phenotype in resting fibroblasts.3 Overexpression of sonic hedgehog (SHH) induces skin fibrosis, whereas inhibition of hedgehog signalling ameliorates experimental fibrosis.4 Based on these experimental findings, this European Scleroderma Trials and Research group (EUSTAR) multicentre cooperative study investigates whether circulating SHH may serve as a surrogate marker for a more severe disease phenotype in patients with SSc. We decided to evaluate SHH serum levels rather than total circulating SHH. Measurements of SHH serum levels are easy and robust but may not assess particulated SHH in various different extracellular vesicles. … |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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