The matriptase-prostasin proteolytic cascade in dermatologic diseases

The proper development and function of skin and hair are dependent on proteolytic activities. Specifically, the matriptase-prostasin cascade is a series of proteolytic reactions in the epidermis integral to normal regulation of desquamation. An increasing amount of research describing this pathway has recently become available, and the importance of this pathway is exhibited by the association of genetic defects in this pathway with human diseases of the skin and hair. Given the relevance of this pathway to dermatology, we provide a review of the current understanding of its relevance to distinct clinical entities, including ichthyosis-hypotrichosis and Netherton syndromes.