Interplay of Staphylococcal and Host Proteases Promotes Skin Barrier Disruption in Netherton Syndrome

• DOI: 10.1016/j.celrep.2020.02.021
• Publication Date: 2020-03-03T16:53:09Z
• Authors: Michael R. Williams; Laura Cau; Yichen Wang; Drishti Kaul; James A. Sanford; Livia S. Zaramela; Shadi Khalil; Anna M. Butcher; Karsten Zengler; Alexander R. Hors...; Christopher L. Du...; Alain Hovnanian; Richard L. Gallo
• Keywords: Skin Barrier

Netherton syndrome (NS) is a monogenic skin disease resulting from loss of function lymphoepithelial Kazal-type-related protease inhibitor (LEKTI-1). In this study we examine if bacteria residing on the are influenced by LEKTI-1 and interaction between human gene resident contributes to disease. Shotgun sequencing microbiome demonstrates that lesional NS subjects dominated Staphylococcus aureus (S. aureus) epidermidis epidermidis). Isolates either species able induce inflammation barrier damage mice. These microbes promote in setting deficiency due excess proteolytic activity promoted S. phenol-soluble modulin α as well increased bacterial proteases staphopain A B or EcpA epidermidis. findings demonstrate critical need for maintaining homeostasis host microbial prevent