Corneodesmosomes And Corneodesmosin: From The Stratum Corneum Cohesion To The Pathophysiology Of Genodermatoses

Corneodesmosin (CDSN) was identified as a key protein in corneodesmosomes, crucial for the cohesion of the stratum corneum and the integrity of hair follicles. It was found in the epidermis, hard palate epithelium, and hair follicles, synthesized by granular keratinocytes. CDSN's adhesive properties were linked to its N-terminal glycine-rich domain, and its dysfunction led to severe epidermal and hair follicle issues in mice. While CDSN's role in psoriasis remained unclear, it was implicated in two monogenic diseases: hypotrichosis simplex of the scalp, where mutated CDSN caused amyloid deposits, and peeling skin disease, characterized by abnormal desquamation and increased allergen penetration due to stratum corneum dyscohesion.