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免疫球蛋白轻链恒定区的片段是AL淀粉样蛋白的组成成分。

Fragments of the constant region of immunoglobulin light chains are constituents of AL-amyloid proteins.

作者信息

Olsen K E, Sletten K, Westermark P

机构信息

Division of Molecular and Immunological Pathology, Linköping University, Sweden.

出版信息

Biochem Biophys Res Commun. 1998 Oct 20;251(2):642-7. doi: 10.1006/bbrc.1998.9508.

Abstract

Immunoglobulin light chains are the precursor proteins of AL-amyloidosis. In the fibril formation process properties of the variable part of the immunoglobulin light chains are believed to be of major importance. In this work it is shown that fragments of the constant part of the immunoglobulin light chain are a constituent of the AL-amyloid proteins of kappa type. A specific antiserum has identified these fragments in gel filtration fractions where the absorbance approached the base line after the main retarded peak. The fragments are small and have been overlooked previously in the purification process. The significance of the constant part in AL-proteins is unclear, but adds new aspects to the discussion of pre- or post-fibrillogenic cleavage of the immunoglobulin light chains.

摘要

免疫球蛋白轻链是淀粉样轻链蛋白(AL-淀粉样变性)的前体蛋白。在原纤维形成过程中,免疫球蛋白轻链可变区的特性被认为至关重要。本研究表明,免疫球蛋白轻链恒定区的片段是κ型AL-淀粉样蛋白的组成部分。一种特异性抗血清已在凝胶过滤组分中鉴定出这些片段,在主要滞留峰之后吸光度接近基线处。这些片段很小,在纯化过程中此前一直被忽视。恒定区在AL蛋白中的意义尚不清楚,但为免疫球蛋白轻链原纤维形成前或形成后切割的讨论增添了新的内容。

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