Keratinocyte adhesion and the missing link: from Dowling-Meara to Hay-Wells. St John's Hospital Dermatological Society Annual Oration 2000.
Maintaining a protective barrier against the environment is an essential function of normal skin. Critical to this role are several structural proteins and glycoproteins that contribute to adhesive junctions linking adjacent keratinocytes and basal keratinocytes to the underlying dermis, as well as other regulatory proteins involved in aspects of epidermal development, differentiation and proliferation. Inherited abnormalities in the genes that encode these components may give rise to a range of genodermatoses, many of which are characterized structurally by a 'missing' or perturbed adhesive link and clinically by congenital skin blistering. This oration reviews some of the original clinical descriptions and observations made in this field, as well as providing an update on the corresponding recent molecular discoveries. The emphasis is on contributions made by past and present members of the St John's Hospital Dermatological Society.
McGrath
J A
JA
Department of Cell and Molecular Pathology, St John's Institute of Dermatology, The Guy's, King's College, and St Thomas' Hospitals' Medical School (GKT), London, UK. john.mcgrath@kcl.ac.uk
D002448:Cell Adhesion / Q000235:genetics; D003896:Desmosomes / Q000648:ultrastructure; D004820:Epidermolysis Bullosa / Q000235:genetics / Q000473:pathology; D022002:Hemidesmosomes / Q000648:ultrastructure; D006801:Humans; D015603:Keratinocytes / Q000473:pathology*; D012868:Skin Abnormalities / Q000235:genetics / Q000473:pathology*