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Item type:Publication, Paw Skin as a Translational Model for Investigating Fibrotic and Inflammatory Wound Healing Defects in Recessive Dystrophic Epidermolysis Bullosa(MDPI AG, 2025-04-30); ; Recessive dystrophic epidermolysis bullosa (RDEB) is a severe genetic disease caused by COL7A1 mutations. It leads to skin fragility, chronic inflammation, and impaired wound healing. The condition often results in fibrotic scarring, pseudosyndactyly, and cutaneous squamous cell carcinoma (SCC). However, current animal models fail to fully replicate chronic RDEB wounds. In this study, we used Collagen VII-hypomorphic mice (Col7a1flNeo/flNeo) and created full-thickness wounds on their paw skin, an area prone to fibrosis due to mechanical stress. We analyzed the healing process using histology, immunofluorescence, and electron microscopy. The RDEB mice showed delayed wound closure, increased inflammation, and poor granulation tissue formation. At 30 days post-injury, we observed persistent fibrosis, with elevated levels of Collagen I, α-SMA+ myofibroblasts, and tenascin-C. These mice also had fewer intraepidermal nerve fibers, which may help explain the neuropathic pain associated with RDEB. Our model reproduces the main features of chronic RDEB wounds. It offers a useful tool for evaluating therapies aimed at reducing inflammation, fibrosis, and tumor risk in these patients.Scopus© Citations 3 4 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, From cadaveric donation to cryopreserved total skin allografts: Transforming the Chilean skin donation model(Elsevier BV, 2025-08)2 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Tiña negra (tinea nigra): comunicación de un caso alóctono en Chile(2013); 1Scopus© Citations 7 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, 1Scopus© Citations 1 1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Atrophic violaceous plaques as the first manifestation of a disorder of GNAS inactivation(2023)1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Scopus© Citations 22 1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Characterisation of the pathophysiology of neuropathy and sensory dysfunction in a mouse model of recessive dystrophic epidermolysis bullosa(2022)32Scopus© Citations 7 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, 1Scopus© Citations 9 1 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Characterization of the epidermal-dermal junction in hiPSC-derived skin organoids(2022); 26Scopus© Citations 46 - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Review of techniques useful for the assessment of sensory small fiber neuropathies: Report from an IFCN expert group(2022)33Scopus© Citations 45