Article
KLK5 and KLK7 Ablation Fully Rescues Lethality of Netherton Syndrome-Like Phenotype.
PLoS genetics - 1 Jan 2017
Kasparek Petr, Ileninova Zuzana, Zbodakova Olga, Kanchev Ivan, Benada Oldrich, Chalupsky Karel, Brattsand Maria, Beck Inken M, Sedlacek Radislav
Abstract excerpt
Netherton syndrome (NS) is a severe skin disease caused by the loss of protease inhibitor LEKTI, which leads to the dysregulation of epidermal proteases and severe skin-barrier defects. KLK5 was proposed as a major protease in NS pathology, however its inactivation is not sufficient to rescue the lethal phenotype of LEKTI-deficient mice. In this study, we further elucidated the in vivo roles of the epidermal...
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