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首页> 外文OA文献 >Amino-terminal identity of co-existent amyloid and non-amyloid immunoglobulin κ light chain deposits. A human disease to study alterations of protein conformation
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Amino-terminal identity of co-existent amyloid and non-amyloid immunoglobulin κ light chain deposits. A human disease to study alterations of protein conformation

机译：淀粉样蛋白和非淀粉样蛋白免疫球蛋白κ轻链沉积物的氨基末端同一性。研究蛋白质构象变化的人类疾病

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摘要

Tissue deposition of monoclonal immunoglobulin light chains is a serious complication in some patients with B cell proliferative disorders. The deposits are typically fibrillar and Congophilic in amyloid (AL) and non-fibrillar and Congophobic in light chain deposition disease (LCDD), and rarely coexist in the same patient. From post-mortem tissue of an individual with fibrillar and non-fibrillar κ light chain deposits in different sites, we separately extracted and analysed biochemically and immunochemically the non-amyloid deposits from isolated glomeruli, the amyloid from isolated renal arteries and the amyloid from myocardium in which the only deposits were amyloid restricted to mural arteries. Western blotting analysis of both the extracted amyloid and the non-amyloid deposits demonstrated 25-kD bands immunoreactive with anti-κ antibody, and the identity of the N-terminal amino acid sequences that belong to the variable region κIV light chain subgroup. This is the first human disease in which antigenically similar but morphologically different deposits have been separately biochemically analysed. We propose that combined LCDD and AL is an ideal human disease to study the relationships and the factors that influence the conversion of non-amyloidogenic to amyloidogenic conformations.
机译：单克隆免疫球蛋白轻链的组织沉积在某些B细胞增生性疾病患者中是严重的并发症。沉积物通常在淀粉样蛋白（AL）中为原纤维状和嗜孔性，在轻链沉积疾病（LCDD）中为非原纤维性和刚果灰，并且很少在同一患者中共存。从在不同部位有原纤维和非原纤维κ轻链沉积的个体的尸体组织中，我们分别提取和生化和免疫化学分析分离肾小球的非淀粉样沉积物，分离肾动脉的淀粉样蛋白和心肌的淀粉样蛋白其中唯一的沉积物是淀粉样蛋白，仅限于壁动脉。提取的淀粉样蛋白和非淀粉样蛋白沉积物的蛋白质印迹分析均显示了与抗κ抗体具有免疫反应性的25 kD条带，以及属于可变区κIV轻链亚组的N末端氨基酸序列的身份。这是人类第一种疾病，其中已分别通过生物化学方法分析了抗原相似但形态不同的沉积物。我们建议LCDD和AL联合使用是一种理想的人类疾病，以研究影响非淀粉样构象向淀粉样蛋白构象转化的关系和因素。

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