Noël L H, Gubler M C, Bobrie G, Savage C O, Lockwood C M, Grünfeld J P
Département de Néphrologie, Hôpital Necker, Paris, France.
Adv Nephrol Necker Hosp. 1989;18:77-94.
For 15 to 20 years, electron microscopy studies have shown that some hereditary renal diseases are characterized by various abnormalities of the basement membranes. In the nail-patella syndrome, in Alport's syndrome and variants, and in benign familial hematuria, the changes involve the glomerular basement membrane (GBM). In nephronophthisis, the tubular basement membrane (TBM) is affected. These histopathologic features are not uniform. The progress afforded by biochemistry, immunology, and molecular biology allows us to compare these various lesions, to analyze their biochemical characteristics and their antigenicity, and to hypothesize as to the mechanisms involved.
在过去15到20年里，电子显微镜研究表明，一些遗传性肾脏疾病的特征是基底膜存在各种异常。在指甲-髌骨综合征、奥尔波特综合征及其变异型以及良性家族性血尿中，病变累及肾小球基底膜（GBM）。在肾痨中，肾小管基底膜（TBM）受到影响。这些组织病理学特征并不一致。生物化学、免疫学和分子生物学的进展使我们能够比较这些不同的病变，分析它们的生化特性和抗原性，并对其中涉及的机制进行假设。
