Dermal papillary fibroblasts promote persistent granulation tissue formation in junctional EB

ABSTRACT: The skin is composed of multiple fibroblast subpopulations with different functions in homeostasis and repair, but their role in skin diseases is largely unknown. Junctional epidermolysis bullosa (JEB) is a hereditary skin disorder characterised by severe skin fragility and aberrant granulation tissue formation, caused by loss of function variants in basement membrane proteins, including laminin-332. We developed JEB-like organotypic (OT) cultures with distinct fibroblast subpopulations and explored their role in an inducible JEB in vivo disease model, mimicking key features of the human disease. Mechanistically, papillary fibroblasts are highly increased in granulation tissue of blistered JEB skin promoting pathological αvβ6 integrin and TGFβ signalling in JEB keratinocytes. Treatment wi

SUBMITTER: Mohammad Khan 

PROVIDER: S-SCDT-10_1038-S44321-026-00475-9 | biostudies-other |

REPOSITORIES: biostudies-other