Muda A O, Rahimi S, Renieri A, Rizzoni G, Massella L, Faraggiana T
Dipartimento di Medicina Sperimentale e Patologia, Università La Sapienza, Roma, Italy.
J Pathol. 1997 Aug;182(4):465-74. doi: 10.1002/(sici)1096-9896(199708)182:4<465::aid-path465>3.0.co;2-v.
This paper describes the ultrastructural immunolocalization of the alpha 2 chain of collagen IV, laminin, and the amino terminal propeptide of collagen I (N-Pro I) in glomeruli of rapidly frozen, freeze-substituted, and low-temperature-embedded renal biopsy specimens from two cases of Alport disease and from normal kidneys. The alpha 2 chain of collagen IV is present in the whole thickness of the basement membrane in glomeruli of Alport patients, while it is limited to the subendothelial portion of the basement membrane of normal glomeruli. Laminin has the same distribution in both normal and Alport glomeruli, but is apparently more concentrated along the basement membrane of normal glomeruli. N-Pro I is localized in mesangial areas and in the basement membrane in Alport cases, while it is not detected in normal glomeruli. These data suggest complex rearrangements of major constituents of the glomerular basement membrane network and demonstrate early deposition of fibrillary collagen proteins in the matrix before the appearance of banded collagen fibres. This finding could be an indicator of early evolution towards glomerulosclerosis.
本文描述了来自两例阿尔波特综合征患者及正常肾脏的快速冷冻、冷冻置换及低温包埋的肾活检标本肾小球中IV型胶原α2链、层粘连蛋白和I型胶原氨基端前肽（N-Pro I）的超微结构免疫定位。IV型胶原α2链存在于阿尔波特综合征患者肾小球基底膜的全层，而在正常肾小球基底膜中仅限于内皮下部分。层粘连蛋白在正常和阿尔波特综合征肾小球中的分布相同，但在正常肾小球基底膜上明显更集中。N-Pro I在阿尔波特综合征病例中定位于系膜区和基底膜，而在正常肾小球中未检测到。这些数据提示肾小球基底膜网络主要成分的复杂重排，并证明在带状胶原纤维出现之前，纤维状胶原蛋白在基质中早期沉积。这一发现可能是向肾小球硬化早期演变的一个指标。
