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淀粉样变性中的窦周功能性单位。

The perisinusoidal functional unit in amyloidosis.

作者信息

David H, Uerlings I, Reinke P

机构信息

Department of Ultrastructural Pathology and Electron Microscopy, Humboldt University, Berlin, GDR.

出版信息

Exp Pathol. 1987;32(3):153-62. doi: 10.1016/s0232-1513(87)80045-2.

DOI:10.1016/s0232-1513(87)80045-2
PMID:3436396
Abstract

The close morphological and functional relations between the various components of the perisinusoidal functional unit may be demonstrated by combination of ultrastructural results with the latest findings in the context of intra-hepatic synthesis and transformation of amyloid. The precursors of amyloid, synthesised in hepatocytes in the region of the endoplasmic reticulum, lysosomes, and plasma membrane of the vascular pole, are moved into the vascular system together with high-density lipoproteins which are probably identical with lipoprotein vesicles close to plasma membranes and in Disse space, and in the vascular system they are transformed to Serum Amyloid A (SAA). A certain role is probably played by Kupffer cells in polymerisation of amyloid A to fibrils. Growing accumulation of fibrils causes ligature and melt-off of peripheral parts from the vascular pole of hepatocytes, with its volume being substantively reduced, so that the spacing between plasma membrane and nucleus may reduce from something between 10 and 12 micron to below 1 micron. There is neither any compression of cytoplasm nor translocation of organelles. Hence, the process can be interpreted as a specific form of atrophy.

摘要

肝窦周功能单位各组成部分之间紧密的形态学和功能关系,可以通过将超微结构结果与肝内淀粉样蛋白合成及转化背景下的最新发现相结合来证明。淀粉样蛋白的前体在血管极内质网、溶酶体和质膜区域的肝细胞中合成,与高密度脂蛋白一起进入血管系统,这些高密度脂蛋白可能与靠近质膜和狄氏间隙中的脂蛋白小泡相同,在血管系统中它们转化为血清淀粉样蛋白A(SAA)。库普弗细胞可能在淀粉样蛋白A聚合成纤维的过程中发挥一定作用。纤维的不断积累导致肝细胞血管极外周部分的结扎和脱落,其体积大幅减小,以至于质膜与细胞核之间的间距可能从10至12微米之间减小到1微米以下。既没有细胞质的压缩,也没有细胞器的移位。因此,该过程可被解释为一种特定形式的萎缩。

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1
The perisinusoidal functional unit in amyloidosis.淀粉样变性中的窦周功能性单位。
Exp Pathol. 1987;32(3):153-62. doi: 10.1016/s0232-1513(87)80045-2.
2
Experimental amyloidosis. Role of the hepatocytes and Kupffer cells in amyloid formation.实验性淀粉样变性。肝细胞和库普弗细胞在淀粉样蛋白形成中的作用。
Appl Pathol. 1985;3(1-2):78-87.
3
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Am J Pathol. 1973 Oct;73(1):97-114.
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The concept of the "perisinusoidal functional unit" of the liver--importance to pathological processes.
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[Morphogenesis of amyloidosis].[淀粉样变性的形态发生]
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Acta Pathol Jpn. 1976 May;26(3):357-66. doi: 10.1111/j.1440-1827.1976.tb00885.x.
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Shedding of peripheral cytoplasm - a mechanism of liver cell atrophy in human amyloidosis.外周细胞质脱落——人类淀粉样变性中肝细胞萎缩的一种机制。
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Pathological study on amyloidosis--scanning electron microscopic observation of amyloid-laden mouse liver.淀粉样变性的病理学研究——载有淀粉样蛋白的小鼠肝脏的扫描电子显微镜观察
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10
Serum amyloid A forms stable oligomers that disrupt vesicles at lysosomal pH and contribute to the pathogenesis of reactive amyloidosis.血清淀粉样蛋白 A 形成稳定的寡聚体,在溶酶体 pH 下破坏囊泡,并有助于反应性淀粉样变性的发病机制。
Proc Natl Acad Sci U S A. 2017 Aug 8;114(32):E6507-E6515. doi: 10.1073/pnas.1707120114. Epub 2017 Jul 25.