C3b灭活剂（KAF）浓度对血清支持补体激活能力的影响。
The influence of C3b inactivator (KAF) concentration on the ability of serum to support complement activation.
作者信息
Lachmann P J, Halbwachs L
出版信息
Clin Exp Immunol. 1975 Jul;21(1):109-14.
Abstract
Minor elevation of the concentration of the C3b inactivator (KAF) in whole serum (by 15-25%) markedly inhibits the capacity of the serum to support complement activation by inulin, aggregated IgG and by low concentrations of CVF. However, there was no effect when large concentrations of CVF were used nor was the spontaneous ageing of C3 slowed by increased KAF concentrations. These findings show that the activity of the C3b feedback cycle can be reduced by raising the KAF concentration above physiological levels. This finding may provide a mechanism for damping down complement activation locally in vivo. It seems that the spontaneous ageing of C3 in vitro does not involve a KAF-inhibitable step and therefore cannot be involved to explain the 'C3 tickover' in vitro.
摘要
全血清中C3b灭活因子（KAF）浓度的轻微升高（升高15%-25%）会显著抑制血清支持菊粉、聚合IgG以及低浓度眼镜蛇毒因子（CVF）激活补体的能力。然而，使用高浓度CVF时则无此效应，而且KAF浓度升高也不会减缓C3的自然衰变。这些发现表明，将KAF浓度提高到生理水平以上可降低C3b反馈循环的活性。这一发现可能为体内局部抑制补体激活提供一种机制。体外C3的自然衰变似乎不涉及KAF可抑制的步骤，因此无法用其来解释体外的 “C3周转”。
相似文献
1
The influence of C3b inactivator (KAF) concentration on the ability of serum to support complement activation.C3b灭活剂（KAF）浓度对血清支持补体激活能力的影响。
Clin Exp Immunol. 1975 Jul;21(1):109-14.
2
3
The effect of anticomplementary substances on properdin in normal and C2-deficient sera.抗补体物质对正常血清和C2缺陷血清中备解素的影响。
Clin Exp Immunol. 1975 Mar;19(3):435-44.
4
Control of C1 activation by nascent C3b and C4b: a mechanism of feedback inhibition.新生C3b和C4b对C1激活的控制：一种反馈抑制机制。
J Immunol. 1986 May 1;136(9):3378-83.
5
A second case of human C3b inhibitor (KAF) deficiency.第二例人类C3b抑制剂（KAF）缺乏症。
Clin Exp Immunol. 1977 Jan;27(1):23-9.
6
C3 metabolism in a patient with deficiency of the second component of complement (C2) and discoid lupus erythematosus.一名补体第二成分（C2）缺乏且患有盘状红斑狼疮患者的C3代谢
Clin Exp Immunol. 1976 May;24(2):238-48.
7
C3b inactivator in the rheumatic diseases. Measurement by radial immunodiffusion and by inhibition of formation of properdin pathway C3 convertase.风湿性疾病中的C3b灭活剂。通过放射免疫扩散法以及对备解素途径C3转化酶形成的抑制作用进行测量。
J Clin Invest. 1976 Jun;57(6):1554-63. doi: 10.1172/JCI108426.
8
The cofactors required by C3 nephritic factor to generate a C3 convertase in vitro.C3肾炎因子在体外生成C3转化酶所需的辅助因子。
Clin Exp Immunol. 1976 Jun;24(3):474-82.
9
Inhibition of immune precipitation by complement.补体对免疫沉淀的抑制作用。
J Immunol. 1984 Sep;133(3):1464-70.
10
Complete absence of the third component of complement in man.人类补体第三成分完全缺失。
J Clin Invest. 1975 Sep;56(3):703-10. doi: 10.1172/JCI108141.
引用本文的文献
1
High prevalence of the hotspot complement factor I p.Ile357Met pathogenic variant in Tunisian atypical hemolytic uremic syndrome patients: report of three new cases and review of the literature.突尼斯非典型溶血性尿毒症综合征患者中热点补体因子I p.Ile357Met致病变体的高流行率：三例新病例报告及文献综述
Front Immunol. 2025 Aug 14;16:1623432. doi: 10.3389/fimmu.2025.1623432. eCollection 2025.
2
Comprehensive functional characterization of complement factor I rare variant genotypes identified in the SCOPE geographic atrophy cohort.全面功能表征在 SCOPE 地图萎缩队列中鉴定的补体因子 I 罕见变异基因型。
J Biol Chem. 2024 Jul;300(7):107452. doi: 10.1016/j.jbc.2024.107452. Epub 2024 Jun 7.
3
Physiology and pathology of the C3 amplification cycle: A retrospective.C3 扩增循环的生理学和病理学：回顾。
Immunol Rev. 2023 Jan;313(1):217-224. doi: 10.1111/imr.13165. Epub 2022 Nov 21.
4
Protein therapeutics and their lessons: Expect the unexpected when inhibiting the multi-protein cascade of the complement system.蛋白类药物及其启示：在抑制补体系统的多蛋白级联反应时，要预料到意料之外的情况。
Immunol Rev. 2023 Jan;313(1):376-401. doi: 10.1111/imr.13164. Epub 2022 Nov 18.
5
Contribution of animal models to the mechanistic understanding of Alternative Pathway and Amplification Loop (AP/AL)-driven Complement-mediated Diseases.动物模型对补体介导的疾病的替代途径和扩增环（AP/AL）驱动的机制理解的贡献。
Immunol Rev. 2023 Jan;313(1):194-216. doi: 10.1111/imr.13141. Epub 2022 Oct 6.
6
The complement alternative pathway in paroxysmal nocturnal hemoglobinuria: From a pathogenic mechanism to a therapeutic target.阵发性睡眠性血红蛋白尿症中的补体替代途径：从发病机制到治疗靶点。
Immunol Rev. 2023 Jan;313(1):262-278. doi: 10.1111/imr.13137. Epub 2022 Sep 15.
7
Interactions Between Pathogenic and the Complement System: A Review of Potential Immune Evasion Mechanisms.病原体与补体系统的相互作用：潜在免疫逃避机制的综述。
Front Cell Infect Microbiol. 2021 Sep 30;11:701362. doi: 10.3389/fcimb.2021.701362. eCollection 2021.
8
How we('ll) treat paroxysmal nocturnal haemoglobinuria: diving into the future.我们将如何治疗阵发性睡眠性血红蛋白尿症：深入未来。
Br J Haematol. 2022 Jan;196(2):288-303. doi: 10.1111/bjh.17753. Epub 2021 Aug 5.
9
Professor Sir Peter J. Lachmann, FRS, FMedSci (1931-2020).彼得·J·拉赫曼爵士教授，皇家学会会员，医学科学院院士（1931 - 2020）
Viruses. 2021 May 28;13(6):1012. doi: 10.3390/v13061012.
10
Acquired decrease of the C3b/C4b receptor (CR1, CD35) and increased C4d deposits on erythrocytes from ICU COVID-19 patients.从 ICU COVID-19 患者的红细胞上获得的 C3b/C4b 受体 (CR1、CD35) 减少和 C4d 沉积增加。
Immunobiology. 2021 May;226(3):152093. doi: 10.1016/j.imbio.2021.152093. Epub 2021 May 9.
本文引用的文献
1
ANTIGEN-ANTIBODY CROSSED ELECTROPHORESIS.抗原-抗体交叉电泳
Anal Biochem. 1965 Feb;10:358-61. doi: 10.1016/0003-2697(65)90278-2.
2
3
Deficiency of C3 inactivator in man.人类C3灭活因子缺乏症。
J Immunol. 1971 Jul;107(1):19-27.
4
Two anticomplementary factors in cobra venom: hemolysis of guinea pig erythrocytes by one of them.眼镜蛇毒中的两种抗补体因子：其中一种可使豚鼠红细胞发生溶血。
J Immunol. 1969 Nov;103(5):944-52.
5
6
The alternate pathway of complement activation. The role of C3 and its inactivator (KAF).补体激活的替代途径。C3及其灭活剂（KAF）的作用。
Immunology. 1973 Feb;24(2):259-75.
7
Inactivator of the third component of complement as an inhibitor in the properdin pathway.补体第三成分灭活剂作为备解素途径中的一种抑制剂。
Proc Natl Acad Sci U S A. 1972 Oct;69(10):2910-3. doi: 10.1073/pnas.69.10.2910.
8
Further studies on the C3b inactivator or conglutinogen activating factor (KAF).关于C3b灭活因子或胶固素原激活因子（KAF）的进一步研究。
Immunochemistry. 1973 Oct;10(10):695-700. doi: 10.1016/0019-2791(73)90213-9.
9
Reaction mechanism of the alternative pathway of complement fixation.补体固定替代途径的反应机制。
Lancet. 1973 Mar 3;1(7801):465-7. doi: 10.1016/s0140-6736(73)91886-2.
10
C3 proactivator convertase and its mode of action.C3 前活化剂转化酶及其作用方式。
J Exp Med. 1972 Apr 1;135(4):1003-8. doi: 10.1084/jem.135.4.1003.
Zotero 插件