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正常人和纯合子C3缺陷患者的单核细胞在体外合成补体第三成分(C3)。

Biosynthesis of the third component of complement (C3) in vitro by monocytes from both normal and homozygous C3-deficient humans.

作者信息

Einstein L P, Hansen P J, Ballow M, Davis A E, Davis J S, Alper C A, Rosen F S, Colten H R

出版信息

J Clin Invest. 1977 Nov;60(5):963-9. doi: 10.1172/JCI108876.

Abstract

Human monocytes synthesized the third component of complement (C3) up to 5 wk in vitro. Evidence for net C3 synthesis was based on (a) incorporation of 14C-labeled amino acids into C3 protein, (b) indentity of the allotype of C3 produced in vitro with that of the doner's serum C3, even in the presence of carrier C3 protein of a different allotype; (c) correspondence of electrophoretic mobility, size, and subunit structure of C3 protein produced in vitro with serum C3; (d) inhibition of C3 production with cycloheximide. Monocytes from two unrelated C3-deficient patients were studied under conditions that supported C3 synthesis by normal monocytes. Serum from each of the patients contained less than 1% of the normal C3 concentration, buth their monocytes produced C3 at approximately equal to 25% of the normal rate when studied after 2 wk in vitro. The C3 produced in vitro by monocytes from one of the patients had the molecular weight of normal serum C3 and dissociated appropriately under reducing conditions. Monocytes from C3-deficient patients could not be distinguished from normals on the basis of morphology, rosetting with C3-coated erythrocytes, or rates of C2, and total protein synthesis.

摘要

人单核细胞在体外可合成补体第三成分(C3)长达5周。C3净合成的证据基于:(a)将14C标记的氨基酸掺入C3蛋白;(b)即使存在不同同种异型的载体C3蛋白,体外产生的C3的同种异型仍与供体血清C3的同种异型相同;(c)体外产生的C3蛋白的电泳迁移率、大小和亚基结构与血清C3一致;(d)用环己酰亚胺抑制C3产生。在支持正常单核细胞合成C3的条件下,对两名无亲缘关系的C3缺陷患者的单核细胞进行了研究。每名患者的血清中C3浓度均低于正常浓度的1%,但在体外培养2周后研究发现,他们的单核细胞产生C3的速率约为正常速率的25%。其中一名患者的单核细胞在体外产生的C3具有正常血清C3的分子量,并且在还原条件下能正确解离。C3缺陷患者的单核细胞在形态、与C3包被红细胞的玫瑰花结形成、C2产生速率以及总蛋白合成方面与正常人无法区分。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cab1/372447/b4ced57d89f0/jcinvest00659-0003-a.jpg

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