Expression of Integrin α6β4 in Junctional Epidermolysis Bullosa

摘要: The integrin α6β4 is a member of the family adhesion receptors. preferentially expressed in stratified squamous epithelia, where it localized hemidesmosomes. A reduced number rudimentary he- midesmosomes often found skin from patients with junctional epidermolysis bullosa (JEB). In this study we have investigated expression specimens (one non-lethal, two lethal) and dystrophic (two) bullosa, using immunofluorescent (IF) staining five different monoclonal antibodies against α6 β4 subunits. intensity IF bullous pemphigoid antigen (BPA) was unreduced along epidermal basement membrane zone (EBMZ) all EB patients, compared to that healthy human controls. However, lethal (Herlitz) JEB, who did not express GB3, BPA showed “stitchy” discontinuous linear pattern EBMZ interruptions at borders adjoining basal keratinocytes. same results were obtained by immunoelectron microscopy. They corresponded freeze-induced partial cell detachment ultimate baso-lateral edge ot keratinocytes JEB skin. lamellipodia location almost completely lacked tonofilaments Furthermore, there split between intra- extracellular epitopes receptor, whereas remains intact salt-split This suggests defect itself or perhaps its ligand.