Murine type VII collagen distorts outcome in human skin graft mouse model for dystrophic epidermolysis bullosa.

Autor: Bremer J; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.; Department of Genetics, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Kramer D; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Eichhorn DS; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Gostyński A; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Diercks GFH; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.; Department of Pathology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Jonkman MF; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., van den Akker PC; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.; Department of Genetics, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Pasmooij AMG; Department of Dermatology, Center of Expertise of Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Jazyk: angličtina
Zdroj: Experimental dermatology [Exp Dermatol] 2019 Oct; Vol. 28 (10), pp. 1153-1155. Date of Electronic Publication: 2018 Aug 20.
DOI: 10.1111/exd.13744
Abstrakt: Human skin graft mouse models are widely used to investigate and develop therapeutic strategies for the severe generalized form of recessive dystrophic epidermolysis bullosa (RDEB), which is caused by biallelic null mutations in COL7A1 and the complete absence of type VII collagen (C7). Most therapeutic approaches are focused on reintroducing C7. Therefore, C7 and anchoring fibrils are widely used as readouts in therapeutic research with skin graft models. In this study, we investigated the expression pattern of human and murine C7 in a grafting model, in which human skin is reconstituted out of in vitro cultured keratinocytes and fibroblasts. The model revealed that murine C7 was deposited in both human healthy control and RDEB skin grafts. Moreover, we found that murine C7 is able to form anchoring fibrils in human grafts. Therefore, we advocate the use of human-specific antibodies when assessing the reintroduction of C7 using RDEB skin graft mouse models.
(© 2018 The Authors. Experimental Dermatology Published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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