Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis.
Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis.
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家族性肌萎缩侧索硬化症中发现的人类 CuZnSOD 突变体三维结构中的亚基不对称性。
DOI：
10.1002/pro.5560070302
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
Eisenberg,D
中科院分区：
文献类型：
--
作者：
Hart,PJ;Liu,H;Pellegrini,M;Nersissian,AM;Gralla,EB;Valentine,JS;Eisenberg,D
在一些遗传性Lou Gehrig病(FALS)患者中发现的人铜/锌超氧化物歧化酶突变体(G37R CuZnSOD)的X射线晶体结构已被确定为1.9？分辨率。这两个SOD亚基在晶体中具有不同的环境，并且在其铜结合部位具有不同的结构。一个亚基(完整的亚基)显示铜离子的四坐标配位几何构型，而另一个亚基(亚单位断裂)显示铜离子的三坐标几何构型。此外，亚单位完整的主链原子的原子位移参数(=30±10ä2)高于亚单位断裂原子的原子位移参数(=24±11ä2)。这种结构是第一个在两个亚基之间显示出较大差异的CuZnSOD。讨论了可能导致这些差异的因素，并建议将较松散的结构与财务会计准则联系起来。
The X‐ray crystal structure of a human copper/zinc superoxide dismutase mutant (G37R CuZnSOD) found in some patients with the inherited form of Lou Gehrig's disease (FALS) has been determined to 1.9 Å resolution. The two SOD subunits have distinct environments in the crystal and are different in structure at their copper binding sites. One subunit (subunitintact) shows a four‐coordinate ligand geometry of the copper ion, whereas the other subunit (subunitbroken) shows a three‐coordinate geometry of the copper ion. Also, subunitintact, displays higher atomic displacement parameters for backbone atoms ( = 30 ± 10 Å2) than subunitbroken ( = 24 ± 11 Å2). This structure is the first CuZnSOD to show large differences between the two subunits. Factors that may contribute to these differences are discussed and a possible link of a looser structure to FALS is suggested.