565 hiPSC-derived skin organoids as tools for disease modelling: characterization of the epidermal-dermal junction

Human induced pluripotent stem cell (hiPSC)-derived hair-bearing skin organoids offer exciting new possibilities for modelling diseases like epidermolysis bullosa. These inherited affect 1 in 30,000 people worldwide and result from perturbed expression and/or structure of components the epidermal-dermal junction, interface between basal keratinocytes epidermis stroma dermis. To establish whether hiPSC-derived might be able to capture salient features bullosa, it is thus important understand developmental stage their junction. this end, we successfully generated three independent hiPSC lines following multistep protocol recently developed by Koehler’s research group thoroughly characterized Using immunofluorescence electron microscopy, showed that adhere laminin-332 type IV collagen-rich basement membrane via I hemidesmosomes integrin β1-based adhesion complexes. Importantly, demonstrated junctions are almost devoid VII collagen, a fibril mediates anchorage This indicates further maturation required take full advantage as disease model some forms particular those caused mutations COL7A1 gene.