KLK5 and KLK7 Ablation Fully Rescues Lethality of Netherton Syndrome-Like Phenotype

The study investigated the roles of epidermal proteases KLK5 and KLK7 in Netherton syndrome (NS) using mouse models. It was found that while the inactivation of either KLK5 or KLK7 alone was insufficient to rescue the lethal phenotype caused by LEKTI deficiency, the simultaneous ablation of both proteases fully rescued the skin barrier defects and postnatal lethality. This allowed the mice to reach adulthood with normal skin and hair growth. The research highlighted that both KLK5 and KLK7 contributed to inflammation and defective differentiation in NS, with KLK7's activity not solely dependent on KLK5. These findings suggested that targeting both KLK5 and KLK7 could be a potential therapeutic strategy for NS.