Molecular basis of complement C3 deficiency in guinea pigs.
Molecular basis of complement C3 deficiency in guinea pigs.
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豚鼠补体 C3 缺乏的分子基础。
DOI：
10.1172/jci114721
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发表时间：
1990
期刊：
影响因子：
--
通讯作者：
Colten,HR
中科院分区：
文献类型：
--
作者：
Auerbach,HS;Burger,R;Dodds,A;Colten,HR
在实验中，以确定遗传决定的第三种成分的补体（C3）在豚鼠的缺乏的生化基础，我们发现，C3缺乏的肝脏和腹腔巨噬细胞含有正常大小（约5 kb）和数量的C3信使RNA，这种mRNA程序的前C3在卵母细胞与肝脏RNA引发和在初级巨噬细胞培养物的合成。在每种情况下，异源二聚体天然C3蛋白以正常动力学分泌，但缺陷细胞的C3蛋白产物未能进行自溶裂解，并且异常地易受蛋白水解的影响。这些数据和选择性失败的C3在血浆中的缺陷动物纳入[14 C]甲胺建议要么突变的C3蛋白质的一级结构或选择性缺陷的共或合成后的加工影响硫酯桥，一个重要的结构C3的功能。通过比较从两个C3缺陷和两个C3充足的豚鼠肝文库产生的C3 cDNA的直接序列分析，排除了C3的一级结构中的突变。鉴别出三个碱基对差异，未导致衍生的氨基酸序列差异。最后，限制性片段长度多态性被确定在C3基因是独立的缺陷表型。C3基因的这个标记允许使用分子生物学和经典遗传学方法来测试这些假设。图片
In experiments to ascertain the biochemical basis of a genetically determined deficiency of the third component of complement (C3) in guinea pigs, we found that C3-deficient liver and peritoneal macrophages contain C3 messenger RNA of normal size (approximately 5 kb) and amounts, that this mRNA programs synthesis of pro-C3 in oocytes primed with liver RNA and in primary macrophage cultures. In each instance, heterodimeric native C3 protein was secreted with normal kinetics but the C3 protein product of the deficient cells failed to undergo autolytic cleavage and was unusually susceptible to proteolysis. These data and a selective failure of C3 in plasma of deficient animals to incorporate [14C]methylamine suggested either a mutation in primary structure of the C3 protein or a selective defect in co- or postsynthetic processing affecting the thiolester bridge, a structure important for C3 function. A mutation in the primary structure of C3 was ruled out by comparison of direct sequence analysis of C3 cDNA generated from two C3 deficient and two C3 sufficient guinea pig liver libraries. Three base pair differences, none resulting in derived amino acid sequence differences were identified. Finally, restriction fragment length polymorphisms were identified in the C3 gene that are independent of the deficiency phenotype. This marker of the C3 gene permits testing of these hypotheses using molecular biological and classical genetic methods.Images
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先天性缺乏补体第三种成分的患者的循环免疫复合物和肾小球肾炎。
影响因子：
158.5
作者：
M. Berger;J. Balow;C. Wilson;M. Frank
通讯作者：
M. Frank
人类完全缺乏补体的第三种成分。
DOI：
10.1172/jci108141
发表时间：
1975
期刊：
The Journal of clinical investigation
影响因子：
--
作者：
M. Ballow;J. Shira;L. Harden;S. Yang;N. Day
通讯作者：
N. Day
影响因子：
4.6
作者：
Ochs,HD;Wedgwood,RJ;Frank,MM;Heller,SR;Hosea,SW
通讯作者：
Hosea,SW
影响因子：
5.4
作者：
BURGER, R;GORDON, J;BITTERSUERMANN, D
通讯作者：
BITTERSUERMANN, D
遗传性补体缺陷对豚鼠体液免疫反应的影响。
影响因子：
4.4
作者：
E. Böttger;T. Hoffmann;U. Hadding;D. Bitter‐Suermann
通讯作者：
D. Bitter‐Suermann