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Impaired lymphoid extracellular matrix impedes antibacterial immunity in epidermolysis bullosa

Nyström, Alexander ; Bornert, Olivier ; Kühl, Tobias ; Gretzmeier, Christine ; Thriene, Kerstin ; Dengjel, Jörn ; Pfister-Wartha, Andrea ; Kiritsi, Dimitra ; Bruckner-Tuderman, Leena

In: Proceedings of the National Academy of Sciences, 2018, vol. 115, no. 4, p. E705–E714

Genetic loss of collagen VII causes recessive dystrophic epidermolysis bullosa (RDEB), a skin fragility disorder that, unexpectedly, manifests also with elevated colonization of commensal bacteria and frequent wound infections. Here, we describe an unprecedented systemic function of collagen VII as a member of a unique innate immune-supporting multiprotein complex in spleen and lymph nodes....