Cell-matrix interactions in cultured dermal fibroblasts from patients with an inherited connective-tissue disorder.

Indirect-immunofluorescence studies were performed on cultured dermal fibroblasts from patients with Pseudoxanthoma Elasticum (PXE), an inherited connective-tissue disorder the pathogenesis of which is still unknown. Apparent abnormalities of cytoskeletal structures were revealed by using phalloidin and specific antibodies to alfa-smooth muscle actin and to vimentin. Altered expression of integrin receptors for different extracellular matrix components seems to be present in the pathological cells as preliminary data suggest by using antibodies against alfa subunits of integrins. This study was designed to test the presence of abnormal cell-matrix interactions responsible for the clinical features and involved in the pathogenesis of the PXE disease.