HB-EGF induces COL7A1 expression in keratinocytes and fibroblasts: possible mechanism underlying allogeneic fibroblast therapy in recessive dystrophic epidermolysis Bullosa.

摘要

Recessive dystrophic epidermolysis bullosa (RDEB) is a mechanobullous disease caused by mutations in the COL7A1 gene that encodes type VII collagen (C7) at the dermal-epidermal junction (DEJ) (Fine ef a/., 2008). The C7 protein is synthesized by both keratinocytes and fibroblasts (Stanley ef a/., 1985). We demonstrated previously that intradermal injections of allogeneic fibroblasts in RDEB can increase C7 expression at the DEJ, although that study did not disclose how long the benefits were sustained for or indicate a therapeutic mode of action (Wong ef a/., 2008). Allogeneic fibroblasts could not be detected 2 weeks after injection but, in some individuals, there was increased C7 protein at the DEJ for at least 3 months