Functional redundancy of extracellular matrix protein 1 in epidermal differentiation

S Sercu, Y Poumay, F Herphelin, J Liekens, L Beek, A Zwijsen, V Wessagowit, D Huylebroeck, J A McGrath, J Merregaert

Research output: Contribution to journalArticlepeer-review

13 Citations (Scopus)


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
Original languageEnglish
Pages (from-to)771 - 775
Number of pages5
JournalBritish Journal of Dermatology
Issue number4
Publication statusPublished - Oct 2007


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