Dysregulation of extracellular matrix and Lysyl Oxidase in Ehlers-Danlos syndrome type IV skin fibroblasts.
Reece FoehrKeith AndersonOwen DombrowskiAnna FoehrErik D FoehrPublished in: Orphanet journal of rare diseases (2024)
Together, these findings suggest dysregulated ECM deposition and processing, reminiscent of a state of fibrosis. Therapeutics that target the dysregulated ECM proteins or help replace damaged tissue may improve clinical outcomes.