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范科尼综合征患者中一种与λ轻链可变结构域相关的独特蛋白质的完整氨基酸序列。

Complete amino acid sequence of a unique protein related to the variable domain of lambda light chain from a case with Fanconi syndrome.

作者信息

Kametani F, Yoshimura K, Tonoike H, Hoshi A, Shinoda T, Isobe T

出版信息

Biochem Biophys Res Commun. 1985 Jan 31;126(2):848-52. doi: 10.1016/0006-291x(85)90262-1.

Abstract

The complete amino acid sequence has been determined of a unique protein from a 55-years-old female with multiple myeloma associated with Fanconi syndrome. It existed in a monomer form with an apparent molecular weight of 10K daltons, and was consisted of 106 amino acid residues. The sequence was characteristic of the V-region of lambda light chains and was highly homologous with that of the first 106 residues of V lambda III subgroup. The presence of an intact light chain as well as a 13K daltons fragment, corresponding to the entire C-region, strongly suggests that the unique component is a catabolic product from the intact light chain rather than an aberrant product of synthesis.

摘要

已确定一名患有与范科尼综合征相关的多发性骨髓瘤的55岁女性体内一种独特蛋白质的完整氨基酸序列。它以单体形式存在,表观分子量为10千道尔顿,由106个氨基酸残基组成。该序列具有λ轻链V区的特征,并且与VλIII亚组的前106个残基高度同源。完整轻链以及对应于整个C区的13千道尔顿片段的存在,强烈表明这种独特成分是完整轻链的分解代谢产物,而不是合成的异常产物。

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