Functional redundancy of extracellular matrix protein 1 in epidermal differentiation
| Autor: | Lisette Beek, Yves Poumay, John A. McGrath, J Merregaert, Danny Huylebroeck, Françoise Herphelin, S Sercu, J Liekens, An Zwijsen, Vesarat Wessagowit |
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| Rok vydání: | 2007 |
| Předmět: |
Adult
Keratinocytes Pathology medicine.medical_specialty Keratin 14 Mice Transgenic ecm1 Dermatology Perlecan transgenic mice Biology Extracellular matrix Extracellular matrix protein 1 Mice expression medicine Animals Humans gene education Involucrin matrix-metalloproteinase-9 Cells Cultured Skin extracellular matrix protein 1 education.field_of_study Extracellular Matrix Proteins integumentary system Epidermis (botany) Cell Differentiation lipoid proteinosis Molecular biology perlecan human keratinocytes medicine.anatomical_structure Mutation biology.protein Immunohistochemistry Lipoid Proteinosis of Urbach and Wiethe Epidermis Keratinocyte epidermal differentiation |
| Zdroj: | The British journal of dermatology. 157(4) |
| ISSN: | 0007-0963 |
| Popis: | Summary Background Extracellular matrix protein 1 (ECM1) is a secreted protein expressed in skin. Its dermatological relevance has been highlighted by the discovery of loss-of-function mutations in ECM1 in patients with lipoid proteinosis (LiP). Objectives To determine the role of ECM1 in epidermal differentiation by examining gene and protein expression of epidermal differentiation markers in individuals with LiP and histological assessment of transgenic mouse skin that overexpresses Ecm1a in basal or suprabasal epidermis. Methods Subconfluent, confluent and postconfluent LiP and control keratinocyte cultures were analysed by Northern and Western blotting for differences in expression of differentiation markers. Expression of these markers was analysed in skin of patients with LiP by immunohistochemistry. To study effects of Ecm1 overexpression on epidermal differentiation, transgenic mice were generated under control of either a keratin 14 or an involucrin promoter. Results No differential expression of the different markers analysed was observed in LiP keratinocytes compared with controls. No histological differences were found in Ecm1-overexpressing mouse skin compared with wild-type. Conclusions Absence of ECM1 does not lead to differences in epidermal differentiation. Moreover, overexpression of Ecm1a in vivo does not exert dramatic effects on epidermal structure. Collectively, these findings suggest no role of ECM1 in epidermal differentiation. |
| Databáze: | OpenAIRE |
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