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Research in practice: Towards deciphering the role of epidermal proteases in recessive dystrophic epidermolysis bullosa progression
Author(s) -
Nauroy Pauline,
Zingkou Eleni,
Sotiropoulou Georgia,
Kiritsi Dimitra
Publication year - 2021
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/ddg.14396
Subject(s) - epidermolysis bullosa , proteases , kallikrein , desquamation , inflammation , fibrosis , medicine , pathology , dermatology , wound healing , cancer research , immunology , biology , biochemistry , enzyme
Summary Recessive dystrophic epidermolysis bullosa (RDEB) is an incurable severe skin disease caused by loss of collagen VII, an extracellular protein that ensures skin cohesion. It manifests in skin blistering and unresolved cycles of wounding and healing that progressively lead to dermal stiffening and early development of aggressive cutaneous squamous cell carcinomas. Inflammation and subsequent tissue fibrosis highly contribute to RDEB pathogenicity and targeting them could provide new therapeutic options. Kallikreins (KLKs) are epidermal secreted proteases, which contribute to skin desquamation and inflammation. Kallikreins are involved in the pathogenesis of several inflammatory skin disorders, but interestingly also in the initiation and progression of different cancers. Our project aims at deciphering the role of KLKs in inflammation, fibrosis, and tumor development in RDEB.