Ljungberg P, Virtanen I, Holmberg C, Jalanko H
Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Finland.
Virchows Arch. 1996 Aug;428(6):333-46. doi: 10.1007/BF00202200.
The aim of this study was to examine the distribution of beta 1 and alpha v integrins (Ints) and some of their ligands in the kidneys of patients with congenital nephrotic syndrome of the Finnish type (CNF) and in controls using indirect immunofluorescence with monoclonal antibodies. The mesangial reactivity of Int alpha 1 and Int beta 1 subunits was more variable and an increased glomerular reactivity with Int alpha 3 and Int-alpha 6 antibodies was found in CNF kidneys than in controls. Int alpha 2 subunit was either completely missing from or found in significantly lesser amounts in CNF kidney glomeruli. The immunoreactivity for Int alpha v was more variable, fainter and also more granular in CNF samples than in control kidneys. The glomerular reactivity for Int beta 5 was more diffuse and weaker, and in sclerotic Bowman's capsules more intense in CNF kidneys than in controls. Immunoreactivity for Int beta 6 was restricted and was comparable in extent in CNF and control kidneys. Of the extracellular matrix components studied, the expression of EDAFn, EDBFn, OncFn, Ln alpha 2 chain, Ln beta 1 chain and tenascin was increased. This is also seen in several glomerular diseases with inflammation and sclerosis. Immunoreactivity for vitronectin was decreased. Several differences were found in the intensity or location of the immunostaining for the beta 1 and alpha v Ints and their ligands in CNF kidneys compared with controls, which have not been found in any other proteinuric disease. Disturbed Int expression pattern in CNF may specifically reflect the disturbance of glomerular function caused by the primary defect in this disease.
本研究的目的是使用单克隆抗体间接免疫荧光法，检测芬兰型先天性肾病综合征（CNF）患者及对照组肾脏中β1和αv整合素（Ints）及其某些配体的分布。与对照组相比，CNF肾脏中Intα1和Intβ1亚基的系膜反应性变化更大，且发现Intα3和Int-α6抗体的肾小球反应性增加。Intα2亚基在CNF肾小体中要么完全缺失，要么含量显著减少。与对照肾脏相比，CNF样本中Intαv的免疫反应性变化更大、更弱且更呈颗粒状。Intβ5的肾小球反应性更弥散、更弱，在硬化的鲍曼囊中，CNF肾脏中的反应性比对照组更强。Intβ6的免疫反应性受限，在CNF和对照肾脏中的程度相当。在所研究的细胞外基质成分中，EDAFn、EDBFn、OncFn、Lnα2链、Lnβ1链和腱生蛋白的表达增加。这在几种伴有炎症和硬化的肾小球疾病中也可见到。玻连蛋白的免疫反应性降低。与对照组相比，CNF肾脏中β1和αv Ints及其配体的免疫染色强度或位置存在一些差异，这些差异在任何其他蛋白尿性疾病中均未发现。CNF中Int表达模式的紊乱可能具体反映了该疾病原发性缺陷所导致的肾小球功能紊乱。
