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- Title
Cystatin M/E knockdown by lentiviral delivery of shRNA impairs epidermal morphogenesis of human skin equivalents.
- Authors
Jansen, Patrick A. M.; van den Bogaard, Ellen H.; Kersten, Ferry F. J.; Oostendorp, Corien; van Vlijmen-Willems, Ivonne M. J. J.; Oji, Vinzenz; Traupe, Heiko; Hennies, Hans C.; Schalkwijk, Joost; Zeeuwen, Patrick L. J. M.
- Abstract
The protease inhibitor cystatin M/E (CST6) regulates a biochemical pathway involved in stratum corneum homeostasis, and its deficiency in mice causes ichthyosis and neonatal lethality. Cystatin M/E deficiency has not been described in humans so far, and we did not detect disease-causing mutations in the CST6 gene in a large number of patients with autosomal recessive congenital ichthyosis, who were negative for mutations in known ichthyosis-associated genes. To investigate the phenotype of CST6 deficiency in human epidermis, we used lentiviral delivery of short hairpin RNAs that target CST6 in a 3D reconstructed skin model. Surprisingly, CST6 deficiency did not cause an ichthyosis-like phenotype, but prevented the development of a multilayered epidermis. From this study, we conclude that CST6 deficiency may be incompatible with normal human foetal development.
- Subjects
PROTEASE inhibitors; CYSTATINS; ICHTHYOSIS; EPIDERMIS; SKIN diseases; MICE
- Publication
Experimental Dermatology, 2012, Vol 21, Issue 11, p889
- ISSN
0906-6705
- Publication type
Article
- DOI
10.1111/exd.12022