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Netherton syndrome: defective kallikrein inhibition in the skin leads to skin inflammation and allergy.

Abstract
Netherton syndrome (NS) is an orphan genetic skin disease with a profound skin barrier defect and severe allergic manifestations. NS is caused by loss of function mutations in SPINK5 encoding lympho-epithelial Kazal-type inhibitor (LEKTI), a secreted multi-domain serine protease inhibitor expressed in stratified epithelia. Studies in mouse models and in NS patients have established that unopposed kallikrein 5 activity triggers stratum corneum detachment and activates PAR-2 signaling, leading to the autonomous production of pro-allergic and pro-inflammatory mediators. This emerging knowledge on NS pathogenesis has highlighted a central role for protease regulation in skin homeostasis but also in the complexity of the disease, and holds the promise of new specific treatments.
AuthorsLaetitia Furio, Alain Hovnanian
JournalBiological chemistry (Biol Chem) Vol. 395 Issue 9 Pg. 945-58 (Sep 2014) ISSN: 1437-4315 [Electronic] Germany
PMID25153381 (Publication Type: Journal Article, Review)
Chemical References
  • Kallikreins
Topics
  • Animals
  • Disease Models, Animal
  • Humans
  • Hypersensitivity (enzymology, pathology)
  • Inflammation (enzymology, pathology)
  • Kallikreins (antagonists & inhibitors, metabolism)
  • Netherton Syndrome (enzymology, pathology, therapy)
  • Skin (pathology)

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