Thursday, March 13, 2014
Publication and contact
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,
All contents Copyright © 1993-2016 BioCentury Publications, Inc. ALL RIGHTS RESERVED. All use of this Web Site and its contents is governed by the BioCentury User Agreement
. The contents of this Web Site are protected under U.S. and foreign copyright and intellectual property laws, and no part of this Web Site or its contents may be photocopied, reproduced or retransmitted in any form without the written consent of BioCentury, which may be requested from Reprints/Permissions
. BioCentury®; The Bernstein Report on BioBusiness™; The BioCentury 100™; The Clear Route to ROI™; Because Real Intelligence is Hard to Find™; BCIQ™; and BioPharma's Knowledge Center™; are trademarks of BioCentury Publications, Inc., P.O. Box 1246, San Carlos, CA 94070. SciBX® and SciBX: Science-Business eXchange® are trademarks of Nature America, Inc. that are jointly used by BioCentury Publications, Inc. and Nature America, Inc.
Thanks for purchasing this article.
Please bookmark this url for future reference.
We have also sent you a confirming e-mail with a receipt and this same url for your
close [ X ]