Premium
Abnormal integrin receptor expression in two cases of familial nephronophthisis
Author(s) -
RAHILLY M.A.,
FLEMING S.
Publication year - 1995
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/j.1365-2559.1995.tb00196.x
Subject(s) - nephronophthisis , expression (computer science) , integrin , medicine , receptor , pathology , endocrinology , biology , genetics , gene , phenotype , computer science , programming language
Familial nephronophthisis is one of the inherited human cystic kidney diseases and is characterized by progressive renal failure. We have investigated abnormalities of cell‐matrix interactions using immunocytochemistry and electron microscopy in three renal biopsies from two patients with familial nephronophthisis and compared our findings to those seen in thirty other renal biopsies. We found expression of the α5 integrin fibronectin receptor in all three samples of nephronophthisis but in no other renal diseases. There was also enhanced expression of the α2 integrin in nephronophthisis but this appeared to be a common response to tubular injury. Electron microscopy showed thickening of the tubular basement membrane and a loss of organization of the basal pole of tubular epithelium. We conclude that altered cell‐substratum adhesion contributes to the pathogenesis of nephronophthisis.