Bence Jones proteins: a powerful tool for the fundamental study of protein chemistry and pathophysiology.
Bence Jones proteins: a powerful tool for the fundamental study of protein chemistry and pathophysiology.
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本斯琼斯蛋白质：蛋白质化学和病理生理学基础研究的强大工具。
DOI：
10.1021/bi00242a001
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发表时间：
1991
期刊：
影响因子：
2.9
通讯作者：
Schiffer,M
中科院分区：
文献类型：
--
作者：
Stevens,FJ;Solomon,A;Schiffer,M
1845年11月1日，亨利·本斯·琼斯博士从沃森博士那里收到了一份标本和附注，附注说：“试管中含有非常高比重的尿液；煮熟后，它变得高度不透明；在加入硝酸的情况下，它会起泡，呈现出红色，变得相当清澈，但当它冷却时，呈现出你所看到的稠度和外观：热量使它重新凝固。这是什么？”(琼斯,1848)。答案并不简单。尽管对后来被称为BenceJones蛋白的物质进行了化学分析，描述了其不寻常的温度依赖性溶解度，并预测了其与现在被称为多发性骨髓瘤的造血肿瘤的潜在诊断相关性，但100多年后，BenceJones蛋白才被确定为人类免疫球蛋白的轻链成分（Edelman & Gaily, 1962）。Bence Jones蛋白通常见于单克隆浆细胞或相关的b细胞免疫增生性疾病患者，如多发性骨髓瘤、轻链相关淀粉样变性（AL），偶尔也见于良性gamopathy、淋巴瘤和白血病。由于它们的单克隆起源和由此产生的化学同质性，以及它们的现成可用性，许多关于免疫球蛋白结构的基本信息最初来自于
Revised Manuscript Received May 17, 1991^) n November 1, 1845, Dr. Henry Bence Jones received from Dr. Watson a specimen and an accompanying note which stated,“The tube contains urine of very high specific gravity; when boiled it becomes highly opake; on the addition of nitric acid it effervesces, assumes a reddish hue, becomes quite clear, but, as it cools, assumes the consistence and appearancewhich you see: heat reliquifiesit. What is it?”(Jones, 1848). The answer was not elementary. Although the substance subse-quently known as Bence Jones protein was chemically ana-lyzed, its unusual temperature-dependent solubility described, and its potential diagnostic relevance for the hematopoietic neoplasm now known as multiple myeloma predicted, more than 100 years passed before BenceJones protein was iden-tified as the light-chain component of human immunoglobulin (Edelman & Gaily, 1962).Bence Jones proteins are typically found inpatients with monoclonal plasma cell or related B-cell immunoproliferative disorders, ie, multiple myeloma, light-chain-associated amyloidosis (amyloidosis AL), and, occasionally, benign gammopathy, lymphoma, and leukemia. Because of their monoclonal origin and resulting chemical homogeneity, as well as their ready availability, much of the fundamental infor-mation on immunoglobulin structure came initially from