Thursday, March 13, 2014
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Transgenic mouse models of
Netherton syndrome expressing human kallikrein-related
peptidase 5 (KLK5)
Transgenic mice expressing
human KLK5 in the epidermis could be used to study the biology of
Netherton syndrome and screen for drugs to treat the indication. Netherton
syndrome is a severe form of ichthyosis caused by mutations in serine
peptidase inhibitor Kazal type 5 (SPINK5;
an inhibitor of KLK5. The transgenic mice survived longer than Spink5-deficient
mice and recapitulated symptoms of Netherton syndrome including reddened,
scaling skin; hyperkeratosis; detachment of the skin's outer epidermal layer
from the underlying granular layer; and skin barrier and hair defects. The
transgenic mice also had enlarged lymph nodes and increased levels of
allergy-associated cells and T helper type 2 (Th2) proinflammatory molecules
in the skin and increased serum levels of IgE compared with wild-type
controls. Future studies could include evaluating potential Netherton
syndrome therapies in the model.
Published online March 13, 2014
Patent and licensing status
Furio, L. et al. J. Exp.
Med.; published online Feb. 17, 2014;
Contact: Alain Hovnanian, University Paris Descartes-Sorbonne,
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