Gubler M C, Levy M, Naizot C, Habib R
Ren Physiol. 1980;3(1-6):405-13. doi: 10.1159/000172790.
Ultrastructural glomerular basement membrane changes are present in most hereditary glomerular diseases: thick and thin basement membrane with splitting of the lamina densa in Alport's syndrome, thin basement membrane in familial benign essential hematuria, and thick basement membrane with the presence of collagen-like fibrils in the nail-patella syndrome. They are useful markers for diagnosis. Moreover, their knowledge has set the problem of the primary biochemical defect in basement membrane metabolism accounting for morphological abnormalities and clinical disturbances. Further biochemical and immunochemical investigations are still required for a better understanding of hereditary glomerular diseases.
阿尔波特综合征中基底膜有厚有薄，且致密层分裂；家族性良性原发性血尿中基底膜薄；指甲-髌骨综合征中基底膜厚且有胶原样纤维。它们是诊断的有用标志物。此外，对它们的了解引发了关于基底膜代谢中导致形态异常和临床紊乱的原发性生化缺陷问题。为了更好地理解遗传性肾小球疾病，仍需要进一步的生化和免疫化学研究。
