Bence Jones proteins and light chains of immunoglobulins. Preferential association of the V lambda VI subgroup of human light chains with amyloidosis AL (lambda).
Bence Jones proteins and light chains of immunoglobulins. Preferential association of the V lambda VI subgroup of human light chains with amyloidosis AL (lambda).
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本斯琼斯蛋白质和免疫球蛋白轻链。
DOI：
10.1172/jci110635
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发表时间：
1982
期刊：
影响因子：
--
通讯作者：
Franklin,EC
中科院分区：
文献类型：
--
作者：
Solomon,A;Frangione,B;Franklin,EC
从患有多发性骨髓瘤和淀粉样变性的患者（SUT）中制备的抗Bonda-Bence Jones蛋白的抗血清对化学定义的可变（V）区Bonda-chain亚组λ VI的Bonda-light链具有特异性。蛋白SUT和其他五个轻链的免疫学确认的V λ VI亚组的序列分析表明，所有六种蛋白质的N-末端序列的原型λ VI蛋白质的特征。的V λ VI亚组的同种型的性质被证明免疫化学：λ VI分子中检测到的轻链分离的IgG蛋白的每个12个正常的个人和λ VI抗原决定簇也检测到完整的IgG蛋白。基于发现91个λ Bence Jones蛋白中有5个属于V λ VI亚组，估计λ VI分子在λ型轻链中的频率约为5%。与其他五种化学分类的λ链亚组的蛋白质相比，V λ VI亚组的蛋白质似乎优先与淀粉样蛋白过程相关，这一事实证明，所有六种λ VI蛋白质均从淀粉样变性AL患者中获得，此外，来自原发性或骨髓瘤患者的42种AIDA型单克隆免疫球蛋白中的5种-相关的淀粉样变性通过免疫扩散分析被分类为具有λ VI型轻链。
An antiserum prepared against a lambda-Bence Jones protein from a patient (SUT) who had multiple myeloma and amyloidosis had specificity for lambda-light chains of the chemically defined variable (V) region lambda-chain subgroup lambda VI. Sequence analyses of protein SUT and of five other lambda-light chains recognized immunologically as of the V lambda VI subgroup revealed that all six proteins had the N-terminal sequence characteristic for prototype lambda VI proteins. The isotypic nature of the V lambda VI subgroup was demonstrated immunochemically: lambda VI molecules were detected among light chains isolated from the IgG proteins of each of 12 normal individuals and lambda VI antigenic determinants were also detectable on the intact IgG proteins. The frequency of lambda VI molecules among lambda-type light chains is estimated to be approximately 5% based on the finding that 5 of 91 lambda Bence Jones proteins were of the V lambda VI subgroup. Proteins of the V lambda VI subgroup, in contrast to those of the other five chemically-classified lambda chain subgroup, appear to be preferentially associated with the amyloid process as evidenced by the fact that all six lambda VI proteins were obtained from patients with amyloidosis AL and, in addition, 5 of 42 lambda-type monoclonal immunoglobulins from patients with primary or myeloma-associated amyloidosis were classified by immunodiffusion analyses as having lambda VI-type light chains.Images