LAMININ, FIBRONECTIN, AND GOODPASTURE ANTIGEN-DETECTION IN PATIENTS WITH ALPORTS-SYNDROME

Indirect immunofluorescence study with laminin and fibronectin monoclo nal antibodies on paraffin sections, as well as with serum from a pati ent with Goodpasture's syndrome with high titer of autoantibodies that recognize the antigenic determinants in human glomerular and tubular basement membrane, was performed on 14 patients with Alport's syndrome and 5 specimens of normal renal tissue obtained from donors in cases of renal transplantation (control group). We found no binding of Goodp asture antigen to glomerular and distal tubular basement membranes in renal biopsy tissue from all 14 patients with Alport's syndrome. In co ntrast, there was bright linear fluorescence of Goodpasture antigen on glomerular and tubular basement membranes of normal renal material. T here was no difference in laminin and fibronectin binding in patients with Alport's syndrome and controls. In all the cases binding was stro ngly positive. These results suggest an abnormality or absence of immu noreactive autoantigen in the glomerular and distal tubular basement m embrane in patients with Alport's syndrome. Therefore, Goodpasture ant igen detection could be an important diagnostic method in early stages of Alport's syndrome when characteristic morphological changes are no t yet developed.