Mar. 5, 2010 Our skin has two crucial barrier functions: it protects against water loss and it prevents penetration of infectious agents and allergens. By studying mice and humans, a team of researchers, led by Alain Hovnanian and colleagues, at CHU Necker-Enfants Malades, France, has now generated data that indicate an important role for the protein elastase 2 (ELA2) in maintaining skin barrier function and suggest that ELA2 might have a role in the development of the rare genetic skin disease Netherton syndrome.
Netherton syndrome is caused by the inefficient inhibition of proteins known as serine proteases, which causes severe skin redness and scaling. In this study, the serine protease ELA2 was found to be expressed in both mouse and human skin cells (keratinocytes). Importantly, ELA2 was found to be hyperactive in skin from patients with Netherton syndrome.
Further, transgenic mice overexpressing ELA2 in the epidermal layer of the skin exhibited cellular abnormalities characteristics of Netherton syndrome and these led to dehydration.
As these data indicate that ELA2 is likely to have an important role in the skin barrier defect seen in patients with Netherton syndrome, the authors suggest that ELA2 might provide a new target for the treatment of Netherton syndrome.
The research appears in the Journal of Clinical Investigation.
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- Chrystelle Bonnart, Céline Deraison, Matthieu Lacroix, Yoshikazu Uchida, Céline Besson, Aurélie Robin, Anaïs Briot, Marie Gonthier, Laurence Lamant, Pierre Dubus, Bernard Monsarrat, and Alain Hovnanian. Elastase 2 is expressed in human and mouse epidermis and impairs skin barrier function in Netherton syndrome through filaggrin and lipid misprocessing. Journal of Clinical Investigation, 2010; DOI: 10.1172/JCI41440
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