Modulating the extracellular matrix to treat wound healing defects in Ehlers-Danlos syndrome.

ABSTRACT: Classic Ehlers-Danlos syndrome (cEDS) is a genetic disorder of the connective tissue that is characterized by mutations in genes coding type V collagen. Wound healing defects are characteristic of cEDS and no therapeutic strategies exist. Herein we describe a murine model of cEDS that phenocopies wound healing defects seen in humans. Our model features mice with conditional loss of Col5a1 in Col1a2 ⁺ fibroblasts (Col5a1CKO). This model shows that an abnormal extracellular matrix (ECM) characterized by fibrillar disarray, altered mechanical properties, and decreased collagen deposition contribute to the wound healing defect. The cEDS animals exhibit decreased expression of epidermal genes and increased inflammation. Finally, we demonstrate that inhibiting mechanosen

SUBMITTER: Kelly-Scumpia KM 

PROVIDER: S-EPMC11389543 | biostudies-literature | 2024 Sep

REPOSITORIES: biostudies-literature