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首页> 外文期刊>Acta crystallographica.Section D. Biological crystallography >Preliminary crystallographic characterization of the human beta2 microglobulin His31Tyr mutant in a tetrameric assembly.
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Preliminary crystallographic characterization of the human beta2 microglobulin His31Tyr mutant in a tetrameric assembly.

机译：的初步晶体特性人类beta2微球蛋白His31Tyr突变四聚物的组装。

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摘要

Patients receiving prolonged haemodialysis treatment are exposed to a variety of arthropathies and bone lesions arising from deposition of amyloid material in the skeletal system. beta2 microglobulin is the 11.7 kDa light chain of the class I major histocompatibility complex, from which it is normally released to plasmatic fluids, transported to kidneys and excreted. Owing to renal failure it accumulates, giving rise to dialysis-related amyloidosis, a severe disease found in patients receiving dialysis for several years. The three-dimensional structure of beta2 microglobulin is known to be based on a seven-stranded beta-sandwich fold, typical of the class C immunoglobulin superfamily. Analysis of the protein fold in different mutants and/or crystal environments and of its structural stability may help in understanding the molecular bases of amyloid fibril formation and of diseases related to protein misfolding. Here, the preliminary crystallographic analysis of the His31Tyr beta2 microglobulin mutant, designed to abolish the copper-ion binding observed in the wild-type protein, is presented. The protein mutant displays increased fold stability, faster folding kinetics and crystallizes in the tetragonal C222(1) space group, with unit-cell parameters a = 105.2, b = 150.2, c = 93.7 A and four molecules per asymmetric unit.
机译：接受长期血液透析的病人接触到各种各样的治疗关节病和骨骼病变引起的在骨骼沉积的淀粉样物质系统。我主要组织相容性链类的复杂,通常是释放血浆的体液,肾脏和运输排泄。引起dialysis-related淀粉样变,严重疾病患者透析了好几年。结构beta2已知微球蛋白基于seven-stranded beta-sandwich折叠,典型的C类免疫球蛋白总科。不同的突变体和/或水晶环境和它的结构稳定性可能帮助了解淀粉样蛋白的分子基础原纤维形成和相关的疾病蛋白质错误折叠。晶体的分析His31Tyr beta2微球蛋白突变,旨在废除在野生型铜离子结合观察蛋白质,。显示褶皱增加稳定性,快速折叠正方的动力学和结晶C222(1)空间群,一个晶胞参数= 105.2, b = 150.2, c = 93.7和4个分子每个不对称单位。

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