Partial amino acid sequence homology between an heredofamilial amyloid protein and human plasma prealbumin.
Partial amino acid sequence homology between an heredofamilial amyloid protein and human plasma prealbumin.
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遗传家族淀粉样蛋白与人血浆前白蛋白之间的部分氨基酸序列同源性。
DOI：
10.1172/jci110114
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
Benson,MD
中科院分区：
文献类型：
--
作者：
Benson,MD
淀粉样原纤维蛋白已被分离出组织的瑞典血统的常染色体显性遗传家族性淀粉样变性。在盐酸胍中溶解后，大量的蛋白质包含在均匀的低分子量部分中。分子量约为14，000，氨基酸分析，双向免疫扩散分析和免疫电泳均支持该物质为前白蛋白相关蛋白。自动序列分析在每个步骤中给出了氨基酸的混合物，表明NH 2-末端是异质的。用溴化氰切割蛋白质后，得到一种同源肽，其序列Val-Val-Val-Leu-Asp-Ala-Val-Arg-Gly-Thr-Pro-对应于用已知的人前白蛋白序列分析的11个位置中的9个，从位置14开始。淀粉样蛋白的抗血清与正常人前白蛋白反应。正常人血清吸收后，该抗血清继续检测淀粉样蛋白患者血清中的决定簇，提示血清前白蛋白异常，这可能是这种遗传性家族性淀粉样变性中原纤维蛋白的前体。对淀粉样变性患者肾组织的间接免疫组化研究显示，抗前白蛋白和抗遗传家族性淀粉样蛋白显着染色，但抗AA或抗κ抗血清未显着染色。该家族与淀粉样变性和葡萄牙家族性淀粉样多发性神经病之间没有遗传关联。图片
Amyloid fibril protein has been isolated from the tissues of a patient of Swedish ancestry with autosomal dominant heredofamilial amyloidosis. After solubilization in guanidine HCl, a significant amount of the protein was contained in a homogeneous low molecular weight fraction. Molecular weight of approximately 14,000, amino acid analysis, double immunodiffusion analysis and immunoelectrophoresis all supported this material being a prealbumin-related protein. Automated sequence analysis gave a mixture of amino acids at each step, suggesting an heterogeneous NH2-terminus. After cleavage of the protein with cyanogen bromide, a homogeneous peptide was obtained with the sequence Val-Val-Val-Leu-Asp-Ala-Val-Arg-Gly-Thr-Pro- corresponding in 9 of the 11 positions analyzed with the known sequence of human prealbumin, starting with position 14. Antiserum raised to the amyloid protein reacted with normal human prealbumin. After absorption with normal human serum, this antiserum continued to detect a determinant in the amyloid patient's serum, suggesting an abnormal serum prealbumin, which may be the precursor of the fibril protein in this type of heredo-familial amyloidosis. Indirect immunohistochemical studies on kidney tissue from the patient with amyloidosis showed marked staining with anti-prealbumin and anti-heredofamilial amyloid protein, but not with anti-AA or anti-kappa antisera. No genetic association between this family and amyloidosis and Portuguese families with familial amyloid polyneuropathy is known.Images