β2微球蛋白与λ轻链在非透析相关浆细胞发育异常相关性系统性淀粉样变性的淀粉样纤维中共存。

Coexistence of beta2 microglobulin and lambda light chain in amyloid fibrils of dialysis-unrelated plasma cell dyscrasia-associated systemic amyloidosis.

作者信息

Fujimoto N, Wada N, Akiyama M, Tajima S, Ishibashi A, Miyakawa S

机构信息

Department of Dermatology, National Defense Medical College, Saitama, Japan.

出版信息

Br J Dermatol. 2002 Sep;147(3):549-53. doi: 10.1046/j.1365-2133.2002.04940.x.

DOI:10.1046/j.1365-2133.2002.04940.x

PMID:12207599

Abstract

BACKGROUND

Systemic amyloidosis occurs as a result of amyloid deposition in various tissues. The amyloid fibrils in systemic amyloidosis have been reported to originate from immunoglobulin light chains.

OBJECTIVE

We studied the composition of amyloid fibrils from two patients with plasma cell-associated systemic amyloidosis (PASA).

METHODS

A double immunofluorescence study of the lesional skin of PASA was undertaken. Amyloid proteins were extracted with distilled water from one case of PASA.

RESULTS

The double immunofluorescence study showed that anti-lambda light chain and anti-beta2 microglobulin antibodies mostly reacted with the same area of amyloid deposit. Amyloid deposits from two patients with PASA who had never undergone haemodialysis showed a positive reaction with the antibodies for beta2 microglobulin as well as immunoglobulin lambda light chain. By the use of immunoblot assay of amyloid fibril proteins, polypeptides immunoreactive with antigamma light chain antibody (29 kDa) and with anti-beta2 microglobulin antibody (12 kDa) were detected.

CONCLUSIONS

These results indicate that beta2 microglobulin is a component of amyloid fibrils in PASA.

摘要

背景

系统性淀粉样变性是由于淀粉样蛋白在各种组织中沉积所致。据报道，系统性淀粉样变性中的淀粉样纤维源自免疫球蛋白轻链。

目的

我们研究了两名浆细胞相关性系统性淀粉样变性（PASA）患者的淀粉样纤维组成。

方法

对PASA患者的病变皮肤进行双重免疫荧光研究。从一例PASA患者中用蒸馏水提取淀粉样蛋白。

结果

双重免疫荧光研究显示，抗λ轻链和抗β2微球蛋白抗体大多与淀粉样沉积物的同一区域发生反应。两名从未接受过血液透析的PASA患者的淀粉样沉积物对β2微球蛋白抗体以及免疫球蛋白λ轻链抗体呈阳性反应。通过对淀粉样纤维蛋白进行免疫印迹分析检测到与抗γ轻链抗体（29 kDa）和抗β2微球蛋白抗体（12 kDa）发生免疫反应的多肽。