Netherton syndrome (NS) is a severe autosomal recessive skin disease characterized by compromised barrier, hair shaft defects, chronic inflammation and allergy. NS caused loss-of-function mutations in the serine peptidase inhibitor Kazal type 5 (SPINK5) gene. Constitutive Spink5 knock-out mice reproduce phenotype, but die within few hours after birth. Here we describe generation of viable, epid...
