Complete primary sequences of two lambda immunoglobulin light chains in myelomas with nonamyloid (Randall-type) light chain deposition disease.
Complete primary sequences of two lambda immunoglobulin light chains in myelomas with nonamyloid (Randall-type) light chain deposition disease.
复制标题
患有非淀粉样蛋白（兰德尔型）轻链沉积病的骨髓瘤中两条 lambda 免疫球蛋白轻链的完整一级序列。
DOI：
10.1016/s0002-9440(10)65573-3
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
Cogné,M
中科院分区：
文献类型：
--
作者：
Decourt,C;Touchard,G;Preud'homme,JL;Vidal,R;Beaufils,H;Diemert,MC;Cogné,M
我们在此报告了负责非淀粉样λ轻链沉积病的λ型单克隆轻链的前两个主要序列（BOU和RAC）。两名患者均患有严重骨髓瘤并发肾衰竭。病理表现通常以沿着肾小管基底膜的刚果红阴性沉积为特征，但与典型的“Randall型”κ轻链沉积疾病略有不同：它们缺乏通常以非淀粉样κ沉积为特征的显著肾小球硬化模式，并与圆柱体或骨髓瘤管型相关。这两种蛋白质序列都是从骨髓浆细胞中相应的互补DNA推导出来的。对于每条链，通过聚合酶链反应的三个独立扩增的产物被测序，发现是相同的。BOU和RAC λ mRNA具有正常的整体结构，由重排为Jλ2Cλ2的Vλ2片段组成，但在其一级序列中显示出许多不寻常的特征。这些取代可能导致轻链构象的变化，从而促进其沿着肾小管基底膜的聚集和沉积。
We herein report on the first two primary sequences (BOU and RAC) of monoclonal light chains of the λ type responsible for nonamyloid λ light chain deposition disease. Both patients were affected with severe forms of myeloma complicated with renal failure. The pathological presentation typically featured Congo red-negative deposits along tubular basement membranes but differed somewhat from the typical “Randall-type” κ light chain deposition disease: they lacked the prominent glomerulosclerosis pattern often featuring nonamyloid κ deposits and were associated with cylinders or myeloma casts. Both protein sequences were deduced from those of the corresponding complementary DNAs in the bone marrow plasma cells. For each chain, products of three independent amplifications by polymerase chain reaction were sequenced and found to be identical. BOU and RAC λ mRNAs had a normal overall structure consisting of Vλ2 segments rearranged to Jλ2Cλ2 but displayed a number of unusual features within their primary sequences. These substitutions are likely responsible for changes in light chain conformation that promote their aggregation and deposition along renal tubule basement membranes.
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DOI：
--
发表时间：
1995
期刊：
影响因子：
--
作者：
尾崎 修治
通讯作者：
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