THE BIOLOGIC SIGNIFICANCE OF AMYLOID *

THE BIOLOGIC SIGNIFICANCE OF AMYLOID *
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淀粉样蛋白的生物学意义 *

DOI:
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发表时间:
1982
影响因子:
5.2
通讯作者:
E. Franklin
E. Franklin
中科院分区:
综合性期刊3区
文献类型:
--
作者:
P. Gorevic;Alice B. Cleveland;E. Franklin

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本讨论的重点将是淀粉样变性作为急性期反应的结果。然而,重要的是要注意,我们所称的“淀粉样疾病”实际上是一组异质性疾病,其特征在于低分子量亚基的聚合形式在组织中沉积,其中许多似乎来源于正常血清蛋白。这些蛋白质中的几种具有或在淀粉样蛋白原纤维中采用主要反平行的β-折叠片构型。这些组织淀粉样蛋白中的一些形式现在可以通过生物化学和免疫组织学来彼此区分,这些生物化学和免疫组织学正在增加临床应用(表1)。AA(所谓的“继发性”)淀粉样变性是一种临床表现多变的疾病,其中组织淀粉样蛋白的主要组分是AA蛋白,推测是源自急性期蛋白SAA的蛋白水解消化产物。许多生物化学上类似于人类AA淀粉样蛋白的动物模型提供了许多关于发病机制中可能重要的因素的信息。还应该指出的是,AA淀粉样变性可能是一种独特的浸润性疾病,可以证明是依赖于,在易感宿主,是一个直接的后果,急性期血浆蛋白反应。
The focus of this discussion will be on amyloidosis as a consequence of the acute phase response. However, it is important to note that “the amyloid diseases,” as we have termed them,’ are in fact a heterogeneous group of disorders characterized by the deposition in tissue of polymeric forms of low molecular weight subunits, many of which appear to be derived from normal serum proteins. Several of these proteins have, or adopt in the amyloid fibril, a predominantly antiparallel beta-pleated sheet configuration.’ Some of these forms of tissue amyloid can now be distinguished from each other by biochemical and immunohistological that are assuming increasing clinical utility (TABLE 1). AA (so-called “secondary”) amyloidosis is a disorder of protean clinician manifestations, in which the major component of tissue amyloid is the AA protein, presumed to be a proteolytic digestion product derived from the acute-phase protein SAA. A number of animal models that are biochemically analogous to human AA amyloid have provided much information as to factors which may be important in pathogenesis.‘ It should also be pointed out that AA amyloidosis is perhaps unique in being an infiltrative disorder that can be shown to be dependent on, and, in susceptible hosts, is a direct consequence of, the acute-phase plasma protein response.
福尔马林固定石蜡切片中淀粉样蛋白的免疫细胞化学鉴定。
DOI: 10.1007/bf00517130
发表时间: 1981
期刊: Histochemistry
影响因子: --
作者:
Shirahama,T;Skinner,M;Cohen,AS
通讯作者: Cohen,AS