Primary structure of a variable region of the VI subgroup (ISE) in light chain deposition disease
Primary structure of a variable region of the VI subgroup (ISE) in light chain deposition disease
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轻链沉积病中 VI 亚组 (ISE) 可变区的一级结构
DOI：
10.1111/j.1365-2249.1993.tb05932.x
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发表时间：
1993
影响因子：
4.6
通讯作者：
M. Cogné
中科院分区：
文献类型：
--
作者：
A. Rocca;A. Khamlichi;P. Aucouturier;L. Noël;L. Denoroy;J. Preud’homme;M. Cogné
尽管单克隆免疫球蛋白轻链（LC）的结构异常被怀疑在非淀粉样蛋白轻链沉积病（LCDD）中起决定性作用，但这种情况在分子水平上的记录很少，因为迄今为止只报道了三个序列。在一例骨髓瘤相关的LCDD病例中，患者的尿液中含有一种未糖基化的κ Bence Jones蛋白，由二聚体和单体组成，表观分子质量为25000，通过N端氨基酸测序将其归属于VκI亚群。利用少量骨髓抽吸获得的材料，采用聚合酶链反应（PCR）扩增恶性浆细胞产生的单克隆κ链的完整可变区序列。从正常大小的κ信使RNA中获得的三个独立扩增的cDNA克隆序列与尿κ链的序列相同。κ mRNA的整体结构是正常的，由VκI序列重排到JκI -可变区域的几个不寻常的特征（LCDD中报道的第一个完整的VκI序列）包括三个替换，在空间靠近的位置引入疏水残基。将N端序列测定与PCR cDNA克隆相结合的策略有助于积累新的序列数据，提高我们对LCDD发病机制的认识。
Although structural abnormalities of monoclonal immunoglobulin light chains (LC) are suspected to play a determinant role in non‐amyloid light chain deposition disease (LCDD), this condition is as yet poorly documented at the molecular level, since only three sequences have been reported to date. In a case of myeloma‐associated LCDD, the patient's urine contained an unglycosylated κ Bence Jones protein made up of dimers and monomers with an apparent molecular mass of 25000 which was assigned to the VκI subgroup by N‐terminal amino acid sequencing. The complete variable region sequence of the monoclonal κ chain produced by the malignant plasma cells was amplified by polymerase chain reaction (PCR) using small amounts of material obtained by bone marrow aspiration. The sequence of three independently amplified cDNA clones derived from a normal‐sized κ messenger RNA was identical to that of the urinary κ chain. The κ mRNA had an overall normal structure made up of a VκI sequence rearranged to JκI‐ Several unusual features of the variable region (the first complete VκI sequence reported in LCDD) included three substitutions that introduced hydrophobic residues at spatially close positions. The strategy associating N‐terminal sequence determination and cDNA cloning by PCR could help in accumulating new sequence data and improving our understanding of LCDD pathogenesis.
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DOI：
10.1016/s0021-9258(18)61070-1
发表时间：
1987-07
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
P. Matsudaira
通讯作者：
P. Matsudaira
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。
影响因子：
2.9
作者：
Stevens,FJ;Solomon,A;Schiffer,M
通讯作者：
Schiffer,M
使用市售气相测序仪进行多肽微序列分析。
影响因子：
2.9
作者：
Esch,FS
通讯作者：
Esch,FS
轻链沉积病源自 kappa I 轻链亚群。
DOI：
--
发表时间：
1989
期刊：
The American journal of pathology
影响因子：
--
作者：
Picken,MM;Frangione,B;Barlogie,B;Luna,M;Gallo,G
通讯作者：
Gallo,G