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LLC小鼠的自发性淀粉样变性

Spontaneous amyloidosis in LLC mice.

作者信息

Chai C K

出版信息

Am J Pathol. 1978 Feb;90(2):381-98.

Abstract

Practically all low leukocyte count (LLC) mice over 1 year of age develop renal amyloidosis. Renal amyloid is deposited in the glomeruli and in the interstitium between the convoluted as well as collecting tubules, with consequent development of cysts and necrosis. LLC mice die of chronic renal failure. Electron microscopic studies reveal amyloid fibrils in the mesangium, a thickening of the basement membranes, and fusion of the foot processes in the glomeruli. Massive amounts of amyloid fibrils are also present in the interstitium, where intracellular fibrils in the fibroblasts as well as in the tubular epithelium cells are found. Vesicles, which are probably formed from membrane disruption, and amorphous materials are seen along the basement membranes. LLC mouse amyloidosis is discussed with regard to its potential as a model for studies on amyloidosis as well as the etiology and origin of amyloid fibrils.

摘要

实际上,所有1岁以上的低白细胞计数(LLC)小鼠都会发生肾淀粉样变性。肾淀粉样物质沉积于肾小球以及曲管和集合管之间的间质中,继而发展为囊肿和坏死。LLC小鼠死于慢性肾衰竭。电子显微镜研究显示,系膜中有淀粉样原纤维,基底膜增厚,肾小球足突融合。间质中也存在大量淀粉样原纤维,在成纤维细胞和肾小管上皮细胞中发现了细胞内原纤维。沿基底膜可见可能由膜破裂形成的囊泡和无定形物质。本文讨论了LLC小鼠淀粉样变性作为淀粉样变性研究模型的潜力以及淀粉样原纤维的病因和起源。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bb33/2018153/751630c20b66/amjpathol00742-0101-a.jpg

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