HUMAN INHIBITOR OF THE 1ST COMPONENT OF COMPLEMENT, C1 - CHARACTERIZATION OF CDNA CLONES AND LOCALIZATION OF THE GENE TO CHROMOSOME-11
HUMAN INHIBITOR OF THE 1ST COMPONENT OF COMPLEMENT, C1 - CHARACTERIZATION OF CDNA CLONES AND LOCALIZATION OF THE GENE TO CHROMOSOME-11
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DOI：
10.1073/pnas.83.10.3161
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发表时间：
1986-05-01
影响因子：
11.1
通讯作者：
ROSEN, FS
中科院分区：
文献类型：
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作者：
DAVIS, AE;WHITEHEAD, AS;ROSEN, FS
C1抑制剂是一种高度糖基化的血浆蛋白，通过灭活丝氨酸蛋白酶亚组分C1 r和C1 s来调节补体第一组分（C1）的活性。已分离出C1抑制剂cDNA克隆，其中一个（pC 1 INH 1，950个碱基对）已部分测序。序列分析表明，C1抑制剂是丝氨酸蛋白酶抑制剂的“超家族”的成员。在测序的区域中，C1抑制剂与抗凝血酶III具有22%的同一性，与α 1-抗胰蛋白酶和α 1-抗胰凝乳蛋白酶具有26%的同一性，与人血管紧张素原具有18%的同一性。C1抑制剂比其他血浆蛋白酶抑制剂具有更大的氨基末端延伸。此外，检查在其他蛋白酶抑制剂中不变的残基表明，C1抑制剂在这些位置中的41个中的14个不同。因此，C1抑制剂似乎在进化的相对早期就从该组中分化出来，尽管可能是在血管紧张素原分化之后。来自正常个体和来自啮齿动物-人类体细胞杂交细胞系（其含有有限但变化的人类染色体补体）的BamHI消化的DNA的Southern印迹分析用于将人类C1抑制剂基因定位于染色体11。
C1 inhibitor is a heavily glycosylated plasma protein that regulates the activity of the first component of complement (C1) by inactivation of the serine protease subcomponents, C1r and C1s. C1 inhibitor cDNA clones have been isolated, and one of these (pC1INH1, 950 base pairs) has been partially sequenced. Sequence analysis demonstrates that the C1 inhibitor is a member of the serpin "superfamily" of protease inhibitors. In the region sequenced, C1 inhibitor has 22% identity with antithrombin III, 26% with alpha 1-antitrypsin and alpha 1-antichymotrypsin, and 18% with human angiotensinogen. C1 inhibitor has a larger amino-terminal extension than do the other plasma protease inhibitors. In addition, inspection of residues that are invariant among the other protease inhibitors shows that C1 inhibitor differs at 14 of 41 of these positions. Thus, it appears that C1 inhibitor diverged from the group relatively early in evolution, although probably after the divergence of angiotensinogen. Southern blot analysis of BamHI-digested DNA from normal individuals and from rodent-human somatic cell hybrid cell lines (that contain a limited but varied human chromosome complement) was used to localize the human C1 inhibitor gene to chromosome 11.