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萨摩耶遗传性肾小球病（SHG）。肾小球毛细血管基底膜分裂的演变。

Samoyed hereditary glomerulopathy (SHG). Evolution of splitting of glomerular capillary basement membranes.

作者信息

Jansen B, Thorner P, Baumal R, Valli V, Maxie M G, Singh A

出版信息

Am J Pathol. 1986 Dec;125(3):536-45.

PMID:3799818
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1888463/
Abstract

Samoyed hereditary glomerulopathy (SHG) in dogs resembles hereditary nephritis (HN) in man. Affected males and carrier females spontaneously develop proteinuria, but only males progress to renal failure. We examined the evolution of splitting of glomerular capillary basement membranes (GCBM) in affected male and carrier female dogs. At birth, examination by light microscopy (LM) in all dogs showed normal glomerular capillaries, but by electron microscopy (EM), many GCBM showed separation of endothelial from visceral epithelial cell basement membranes. By LM, glomerular capillaries of affected males appeared normal for up to 4 months, but then isolated and eventually all capillaries became thickened and split. However, by EM, a bilaminar appearance of the GCBM was seen within 1 month which evolved into multilaminar splitting that became extensive as renal failure ensued. Carrier females showed mild nonprogressive changes in GCBM. These results with SHG permitted speculation on the evolution of splitting of GCBM in human HN.

摘要

犬类的萨摩耶德遗传性肾小球病（SHG）与人类的遗传性肾炎（HN）相似。患病雄性和携带基因的雌性会自发出现蛋白尿，但只有雄性会发展为肾衰竭。我们研究了患病雄性和携带基因的雌性犬类肾小球毛细血管基底膜（GCBM）分裂的演变过程。出生时，所有犬类经光学显微镜（LM）检查显示肾小球毛细血管正常，但经电子显微镜（EM）检查，许多GCBM显示内皮细胞与脏层上皮细胞基底膜分离。经LM检查，患病雄性的肾小球毛细血管在长达4个月的时间里看起来正常，但随后出现孤立现象，最终所有毛细血管都变厚并分裂。然而，经EM检查，在1个月内即可看到GCBM呈现双分层外观，随着肾衰竭的发生，这种双分层演变成多层分裂并变得广泛。携带基因的雌性在GCBM方面表现出轻微的非进行性变化。这些关于SHG的结果有助于推测人类HN中GCBM分裂的演变过程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/80d62b41a14f/amjpathol00153-0130-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/0a33fb6b8002/amjpathol00153-0126-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/cd2d51c15110/amjpathol00153-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/cae5692b7eae/amjpathol00153-0128-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/c8d1149e3174/amjpathol00153-0129-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/80d62b41a14f/amjpathol00153-0130-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/0a33fb6b8002/amjpathol00153-0126-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/cd2d51c15110/amjpathol00153-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/cae5692b7eae/amjpathol00153-0128-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/c8d1149e3174/amjpathol00153-0129-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6905/1888463/80d62b41a14f/amjpathol00153-0130-a.jpg

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本文引用的文献

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Alport's syndrome. A report of 58 cases and a review of the literature.奥尔波特综合征。58例报告及文献综述。
Am J Med. 1981 Mar;70(3):493-505. doi: 10.1016/0002-9343(81)90571-4.
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Absence of nephritogenic GBM antigen(s) in some patients with hereditary nephritis.一些遗传性肾炎患者缺乏致肾炎性肾小球基底膜抗原。
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Early pathologic features of hereditary nephritis: a clinicopathologic correlation.遗传性肾炎的早期病理特征：临床病理相关性
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以犬类和人类作为伴侣模型系统来理解遗传性疾病。
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Transfer of the alpha 5(IV) collagen chain gene to smooth muscle restores in vivo expression of the alpha 6(IV) collagen chain in a canine model of Alport syndrome.将α5（IV）胶原链基因转移至平滑肌可在犬Alport综合征模型中恢复α6（IV）胶原链的体内表达。
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The inner ear of dogs with X-linked nephritis provides clues to the pathogenesis of hearing loss in X-linked Alport syndrome.患有X连锁肾炎的犬类内耳为X连锁遗传性肾炎综合征听力损失的发病机制提供了线索。
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Absence of the alpha6(IV) chain of collagen type IV in Alport syndrome is related to a failure at the protein assembly level and does not result in diffuse leiomyomatosis.阿尔波特综合征中IV型胶原α6(IV)链的缺失与蛋白质组装水平的缺陷有关，且不会导致弥漫性平滑肌瘤病。
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