CELL-MATRIX INTERACTIONS IN CULTURED DERMAL FIBROBLASTS FROM PATIENTSWITH AN INHERITED CONNECTIVE-TISSUE DISORDER

Indirect-immunofluorescence studies were performed on cultured dermal fibroblasts from patients with Pseudoxanthoma Elasticum (PXE), an inhe rited connective-tissue disorder the pathogenesis of which is still un known. Apparent abnormalities of cytoskeletal structures were revealed by using phalloidin and specific antibodies to alfa-smooth muscle act in and to vimentin. Altered expression of integrin receptors for diffe rent extracellular matrix components seems to be present in the pathol ogical cells as preliminary data suggest by using antibodies against a lfa subunits of integrins. This study was designed to test the presenc e of abnormal cell-matrix interactions responsible for the clinical fe atures and involved in the pathogenesis of the PXE disease.