Xeroderma pigmentosum is a rare inherited disorder that impairs the ability to repair the DNA lesions caused by UV light exposure. Currently, there is no curative treatment for these patients, who require continuous sun protection. Our aim is to model the disease and develop treatments that will enhance the quality of life for these patients.
Impact
Modeling skin diseases using a highly controlled stem cell-based system enables the capture of complex disease mechanisms involving interactions among multiple cell types. This understanding is essential for developing clinical interventions that could significantly reduce the burden of these devastating disorders.
reNEW research
Principal investigator Karine Raymond and her team have developed Pluripotent Stem cells that differ only by the gene associated with the disease. By generating hair-bearing skin organoids that either contain or lack the mutation, they can unravel specific complex features associated with the disease and explore treatments that could correct these defects.
Image description
A skin organoid that blooms into a bouquet of green stems (hairs) displaying red flowers (the UV-protective molecule missing in patients) within a rich white soil (fibroblasts) that allows the blue sap (cell nuclei) to nourish the bouquet.
Credits
Myrthe Flesseman, research technician, Karine Raymond Lab, reNEW Leiden, Netherlands.
