Project Title
Validación de una matriz polimérica funcionalizada con secretoma derivado de células madre mesenquimáticas y queratinocitos para el cierre de heridas crónicas y la disminución de la fibrosis en epidermólisis bulosa
Partner Organisations
Internal ID
ID24I10119
Principal Investigator
Type
applied research
Status
VIGENTE
Start Date
March 25, 2024
End Date
March 25, 2026
Organisations
Pontificia Universidad Católica de Chile
Inbiocriotec
DEBRA CHILE
Fields of Science and Technology (OECD)
Medical and Health sciences

Filters

Settings

Now showing 1 - 1 of 1
  • Loading...
    Thumbnail Image
    Some of the metrics are blocked by your 
    consent settings
    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)
    DE GREGORIO CONCHA, CRISTIAN ALEJANDRO  
    ;
    Giselle Ramos-Gonzalez
    ;
    Bernardo Morales-Catalán
    ;
    EZQUER, EDUARDO FERNANDO  
    ;
    EZQUER, EDUARDO MARCELO  
    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