期刊
EXPERIMENTAL DERMATOLOGY
卷 20, 期 8, 页码 689-695出版社
WILEY
DOI: 10.1111/j.1600-0625.2011.01313.x
关键词
collagen; Ehlers-Danlos syndromes; fibrillin; fibrillogenesis; integrins; laminin; Marfan syndrome; skin blistering diseases; TGF-beta
类别
资金
- Deutsche Zentrum fur Luft und Raumfahrt [50WB0721]
- Federal Ministry for Education and research (Networks Epidermolysis bullosa) [01GM0832]
- Stem Cell Therapy for Severe Skin Fragility Syndromes [01GN0972]
- Marga und Boll Stiftung
- Center for Molecular Medicine Cologne
- Collaborative Research Centre 829 at the University of Cologne [SFB 829)]
- Medical Faculty of the University of Cologne
- Centre National de la Recherche Scientifique
The current understanding of the role of extracellular matrix proteins is mainly based on their structural properties and their assembly into complex networks. The multiplicity of interactions between cells, cytokines and growth factors within the networks determines functional units dictating the biophysical properties of tissues. This review focuses on the understanding how alterations in the genes, modifying enzymes or biological functions of extracellular matrix molecules, lead to inborn or acquired skin disorders. Analysis of the disease mechanisms provides the basis for the emerging concept that not solely structural defects of single extracellular matrix proteins are at fault, but rather that the functional unit as a whole is not working properly, causing similar clinical symptoms although the causative genes are entirely different. The understanding of these disease-causing pathways has already led to surprising new therapeutic developments applied to rare inborn disorders. They now permit to design new concepts for the treatment of more common diseases associated with the accumulation of connective tissue and alterations of the biomechanical properties of the extracellular matrix.
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