Engvig J P, Olsen K E, Gislefoss R E, Sletten K, Wahlström O, Westermark P
Department of Biochemistry and Biotechnology Center of Oslo, University of Oslo, Norway.
Scand J Immunol. 1998 Jul;48(1):92-8. doi: 10.1046/j.1365-3083.1998.00352.x.
AL-amyloidoses are generally described as a group of disorders in which N-terminal fragments of monoclonal immunoglobulin light chains are transferred into amyloid fibrils. We have, by amino acid sequence analyses and immunological methods, characterized the Bence-Jones protein and the corresponding AL protein as a kappa III immunoglobulin light chain from material of a patient with systemic AL-amyloidosis presenting as a local inguinal tumour. The two proteins showed some unique features. The major part of the AL amyloid fibril protein consisted of C-terminal fragments of the Bence-Jones protein. Furthermore, both the Bence-Jones protein and the AL protein were glycosylated, with possibly a glycosylation in the constant part of the light chain.
淀粉样轻链蛋白（AL-淀粉样变性）通常被描述为一组疾病，其中单克隆免疫球蛋白轻链的N端片段会转化为淀粉样纤维。我们通过氨基酸序列分析和免疫学方法，从一名表现为局部腹股沟肿瘤的系统性AL淀粉样变性患者的材料中，将本周氏蛋白和相应的AL蛋白鉴定为κIII免疫球蛋白轻链。这两种蛋白表现出一些独特的特征。AL淀粉样纤维蛋白的主要部分由本周氏蛋白的C端片段组成。此外，本周氏蛋白和AL蛋白都进行了糖基化，可能在轻链的恒定区存在糖基化。
