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[淀粉样变性]

[The amyloidosis].

作者信息

Casirola G, Invernizzi R, Ippoliti G, Marini G

出版信息

Minerva Med. 1978 Jul 7;69(33):2217-34.

PMID:355933
Abstract

The clinical and pathological features of amyloidosis are examined in the light of a classification based on anatomical models of amyloid distribution: 1) generalised amyloidosis, including primary and secondary sporadic forms, hereditary and familial amyloidosis, and senile amyloidosis; 2) local amyloidosis, including amyloid tumours, neoplasia with amyloid stroma, and other forms. The nature of amyloid is described. Morphological, ultrastructural, biochemical and immunochemical research has shown that: 1) amyloid is a fibrillar protein with a typical EM and X-ray diffraction appearance; 2) two main types of amyloid proteins exist, one related to Ig light chains and found in primary forms and in association with myeloma, the other (called AA proteins) being the main component of secondary forms and certain types of familial amyloidosis; 3) serum proteins structurally related to the AA proteins may be used as amyloid precursors. The cause and mechanisms of amyloid production are becoming clearer. In particular, a relation with the production of Igs has been established. Improved methods for the diagnosis and treatment of the disease will throw more light and the nature of amyloid and its protein precursors.

摘要

根据基于淀粉样蛋白分布解剖模型的分类,对淀粉样变性的临床和病理特征进行了研究:1)全身性淀粉样变性,包括原发性和继发性散发性形式、遗传性和家族性淀粉样变性以及老年性淀粉样变性;2)局限性淀粉样变性,包括淀粉样瘤、伴有淀粉样间质的肿瘤以及其他形式。描述了淀粉样蛋白的性质。形态学、超微结构、生化和免疫化学研究表明:1)淀粉样蛋白是一种具有典型电子显微镜和X射线衍射外观的纤维状蛋白质;2)存在两种主要类型的淀粉样蛋白,一种与免疫球蛋白轻链相关,见于原发性形式并与骨髓瘤相关,另一种(称为AA蛋白)是继发性形式和某些类型家族性淀粉样变性的主要成分;3)与AA蛋白结构相关的血清蛋白可作为淀粉样蛋白前体。淀粉样蛋白产生的原因和机制正变得更加清晰。特别是,已确定与免疫球蛋白的产生有关。该疾病诊断和治疗方法的改进将更清楚地揭示淀粉样蛋白及其蛋白质前体的性质。

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