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J Invest Dermatol ; 135(3): 759-767, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25347115

ABSTRACT

In healthy human skin host defense molecules such as antimicrobial peptides (AMPs) contribute to skin immune homeostasis. In patients with the congenital disease ectodermal dysplasia (ED) skin integrity is disturbed and as a result patients have recurrent skin infections. The disease is characterized by developmental abnormalities of ectodermal derivatives and absent or reduced sweating. We hypothesized that ED patients have a reduced skin immune defense because of the reduced ability to sweat. Therefore, we performed a label-free quantitative proteome analysis of wash solution of human skin from ED patients or healthy individuals. A clear-cut difference between both cohorts could be observed in cellular processes related to immunity and host defense. In line with the extensive underrepresentation of proteins of the immune system, dermcidin, a sweat-derived AMP, was reduced in its abundance in the skin secretome of ED patients. In contrast, proteins involved in metabolic/catabolic and biosynthetic processes were enriched in the skin secretome of ED patients. In summary, our proteome profiling provides insights into the actual situation of healthy versus diseased skin. The systematic reduction in immune system and defense-related proteins may contribute to the high susceptibility of ED patients to skin infections and altered skin colonization.


Subject(s)
Ectodermal Dysplasia/immunology , Ectodermal Dysplasia/metabolism , Peptides/metabolism , Proteomics , Skin/metabolism , Adult , Animals , Case-Control Studies , Disease Models, Animal , Female , Gene Expression Profiling , Humans , Male , Mice , Mice, Inbred C57BL , Ointments , Peptides/administration & dosage , Peptides/therapeutic use , Staphylococcal Skin Infections/drug therapy , Staphylococcal Skin Infections/microbiology , Staphylococcus aureus , Sweat Glands/metabolism
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