Carone F A, Makino H, Kanwar Y S
Department of Pathology, Northwestern University Medical School, Chicago, Illinois 60611.
Am J Pathol. 1988 Mar;130(3):466-71.
Status of basement membrane antigens in renal polycystic disease was investigated. Antibodies directed against various components of basement membrane, including anti-heparan sulfate proteoglycan, Type IV collagen, laminin, and fibronectin, were employed. Their reactivities with basement membranes of normal and cystic segments of the renal tubules were ascertained by indirect immunofluorescence. The tissues were obtained either from kidneys of patients with adult (autosomal dominant) polycystic disease or from rats with renal cystic change induced by administration of 2-amino-4,5-diphenylthiazole HCl. The human and rat tissues that had undergone cystic change exhibited essentially similar results. A loss of reactivity to anti-heparan sulfate proteoglycan antibodies was observed. The reactivity toward anti-Type IV collagen and laminin either remained unchanged or was focally increased. The reactivity toward fibronectin, normally absent, increased dramatically in the peritubular regions and interstitium. The results indicate that there is an imbalance in various antigenic components associated with renal tubular cystic disease in rat and man, which may have a fundamental role in the pathogenesis of this disorder.
对肾多囊病中基底膜抗原的状态进行了研究。使用了针对基底膜各种成分的抗体，包括抗硫酸乙酰肝素蛋白聚糖、IV型胶原、层粘连蛋白和纤连蛋白。通过间接免疫荧光确定它们与肾小管正常段和囊性段基底膜的反应性。组织取自患有成人（常染色体显性）多囊病患者的肾脏，或取自给予盐酸2-氨基-4,5-二苯基噻唑诱导肾囊性改变的大鼠。发生囊性改变的人和大鼠组织显示出基本相似的结果。观察到对抗硫酸乙酰肝素蛋白聚糖抗体的反应性丧失。对抗IV型胶原和层粘连蛋白的反应性要么保持不变，要么局部增加。正常情况下不存在的对纤连蛋白的反应性在肾小管周围区域和间质中显著增加。结果表明，在大鼠和人类肾小管囊性疾病中，各种抗原成分存在失衡，这可能在该疾病的发病机制中起基本作用。
