In a mouse model of Netherton syndrome (NS) it was demonstrated that this KLK7 ABP could elicit a therapeutic effect in vivo by inhibition of KLK7 activity, which validated the role of KLK7 as drug target for this rare disease and potentially for other common skin diseases, such as atopic dermatitis, in which KLK7 is implicated [16]. Here, KLK7 is linked to Netherton syndrome.