Abnormal integrin receptor expression in two cases of familial nephronophthisis

M A Rahilly; S Fleming

doi:10.1111/j.1365-2559.1995.tb00196.x

Abnormal integrin receptor expression in two cases of familial nephronophthisis

M A Rahilly
, S Fleming

Research output: Contribution to journal › Article › peer-review

19 Citations (Scopus)

Abstract

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 alpha 5 integrin fibronectin receptor in all three samples of nephronophthisis but in no other renal diseases. There was also enhanced expression of the alpha 2 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.

Original language	English
Pages (from-to)	345-9
Number of pages	5
Journal	Histopathology
Volume	26
Issue number	4
DOIs	https://doi.org/10.1111/j.1365-2559.1995.tb00196.x
Publication status	Published - 1995

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10.1111/j.1365-2559.1995.tb00196.x

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title = "Abnormal integrin receptor expression in two cases of familial nephronophthisis",

abstract = "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 alpha 5 integrin fibronectin receptor in all three samples of nephronophthisis but in no other renal diseases. There was also enhanced expression of the alpha 2 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.",

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T1 - Abnormal integrin receptor expression in two cases of familial nephronophthisis

AU - Rahilly, M A

AU - Fleming, S

PY - 1995

Y1 - 1995

N2 - 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 alpha 5 integrin fibronectin receptor in all three samples of nephronophthisis but in no other renal diseases. There was also enhanced expression of the alpha 2 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.

AB - 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 alpha 5 integrin fibronectin receptor in all three samples of nephronophthisis but in no other renal diseases. There was also enhanced expression of the alpha 2 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.

U2 - 10.1111/j.1365-2559.1995.tb00196.x

DO - 10.1111/j.1365-2559.1995.tb00196.x

M3 - Article

C2 - 7607623

SN - 0309-0167

VL - 26

SP - 345

EP - 349

JO - Histopathology

JF - Histopathology

IS - 4

ER -

Abnormal integrin receptor expression in two cases of familial nephronophthisis

Abstract

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