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一名复发性脑膜炎球菌感染患者存在遗传性C8缺陷:C8分子功能失调及与HLA系统无连锁关系的进一步证据

Inherited deficiency of C8 in a patient with recurrent meningococcal infections: further evidence for a dysfunctional C8 molecule and nonlinkage to the HLA system.

作者信息

Densen P, Brown E J, O'Neill G J, Tedesco F, Clark R A, Frank M M, Webb D, Myers J

出版信息

J Clin Immunol. 1983 Jan;3(1):90-9. doi: 10.1007/BF00919144.

DOI:10.1007/BF00919144
PMID:6186685
Abstract

An adult male with recurrent meningococcal infections is reported whose serum lacked functional C8 activity but possessed antigenic C8. The addition of 1500 U of purified C8/ml of serum restored hemolytic activity to normal. Four to five times more C8 was required to restore bactericidal activity than to restore hemolytic activity. Bactericidal activity could also be restored by mixing the patient's serum with a second C8-deficient serum that lacked detectable antigenic or functional C8. The patient's serum contained bactericidal antibody for groups A, B, C, and Y meningococci and specific antibody to group Y capsular polysaccharide. There was two to three times more bactericidal antibody activity in the serum than in a pool of normal sera for the infecting strain. Family studies disclosed a sibling who was HLA identical to the patient but whose serum contained normal amounts of total hemolytic and C8 functional activity.

摘要

据报道,一名成年男性反复发生脑膜炎球菌感染,其血清缺乏功能性C8活性,但含有抗原性C8。每毫升血清中加入1500 U纯化C8可使溶血活性恢复正常。恢复杀菌活性所需的C8量是恢复溶血活性所需量的4至5倍。将患者血清与第二种缺乏可检测到的抗原性或功能性C8的C8缺陷血清混合,也可恢复杀菌活性。患者血清中含有针对A、B、C和Y群脑膜炎球菌的杀菌抗体以及针对Y群荚膜多糖的特异性抗体。血清中的杀菌抗体活性比感染菌株的正常血清池高两到三倍。家族研究发现一名与患者HLA相同的同胞,但其血清中总溶血活性和C8功能活性含量正常。

相似文献

1
Inherited deficiency of C8 in a patient with recurrent meningococcal infections: further evidence for a dysfunctional C8 molecule and nonlinkage to the HLA system.一名复发性脑膜炎球菌感染患者存在遗传性C8缺陷:C8分子功能失调及与HLA系统无连锁关系的进一步证据
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2
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引用本文的文献

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Phagocytic killing and antibody response during the first year after tetravalent meningococcal vaccine in complement-deficient and in normal individuals.四价脑膜炎球菌疫苗接种后第一年,补体缺陷个体与正常个体的吞噬细胞杀伤作用及抗体反应。
J Clin Immunol. 2000 Jan;20(1):46-53. doi: 10.1023/a:1006642611069.
2
Complement deficiency states and meningococcal disease.补体缺陷状态与脑膜炎球菌病
Immunol Res. 1993;12(3):295-311. doi: 10.1007/BF02918259.
3
Inherited C8 beta subunit deficiency in a patient with recurrent meningococcal infections: in vivo functional kinetic analysis of C8.

本文引用的文献

1
Deficiency of C7 with systemic lupus erythematosus: solubilization of immune complexes in complement-deficient sera.C7缺乏与系统性红斑狼疮:补体缺陷血清中免疫复合物的溶解
Arthritis Rheum. 1981 Jan;24(1):87-93. doi: 10.1002/art.1780240114.
2
The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure.人类补体的第八个成分。其异常亚基结构的纯化及理化特性
J Biol Chem. 1980 Dec 25;255(24):11997-2005.
3
Binding of the eighth component of human complement to the soluble cytolytic complex is mediated by its beta subunit.
一名复发性脑膜炎球菌感染患者存在遗传性C8β亚基缺陷:C8的体内功能动力学分析
Clin Exp Immunol. 1985 Apr;60(1):183-90.
4
Complement-induced glomerular epithelial cell injury. Role of the membrane attack complex in rat membranous nephropathy.补体诱导的肾小球上皮细胞损伤。膜攻击复合物在大鼠膜性肾病中的作用。
J Clin Invest. 1986 Apr;77(4):1096-107. doi: 10.1172/JCI112408.
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Complement induces a transient increase in membrane permeability in unlysed erythrocytes.补体可使未裂解红细胞的膜通透性短暂增加。
J Clin Invest. 1988 Aug;82(2):594-600. doi: 10.1172/JCI113637.
6
Ca2+-activated K+ efflux limits complement-mediated lysis of human erythrocytes.钙离子激活的钾离子外流限制补体介导的人红细胞溶解。
J Clin Invest. 1989 May;83(5):1466-71. doi: 10.1172/JCI114039.
7
Infectious diseases associated with complement deficiencies.与补体缺陷相关的传染病。
Clin Microbiol Rev. 1991 Jul;4(3):359-95. doi: 10.1128/CMR.4.3.359.
人补体第八成分与可溶性溶细胞复合物的结合是由其β亚基介导的。
J Biol Chem. 1980 Nov 25;255(22):10579-82.
4
A familial dysfunction of the eight component of complement (C8).补体第八成分(C8)的家族性功能障碍。
Clin Immunol Immunopathol. 1980 Jun;16(2):180-91. doi: 10.1016/0090-1229(80)90202-0.
5
Role of the beta subunit in interaction of the eighth component of human complement with the membrane-bound cytolytic complex.β亚基在人补体第八成分与膜结合溶细胞复合物相互作用中的作用。
J Biol Chem. 1981 Apr 10;256(7):3258-62.
6
Occurrence of an incomplete C8 molecule in homozygous C8 deficiency in man.人类纯合子C8缺乏症中出现不完全C8分子。
J Exp Med. 1981 Nov 1;154(5):1599-607. doi: 10.1084/jem.154.5.1599.
7
Meningococcal meningitis in familial deficiency of the fifth component of complement.补体第五成分家族性缺陷中的脑膜炎球菌性脑膜炎
Pediatrics. 1981 Jun;67(6):882-6.
8
Recurrent meningococcal infections associated with a functional deficiency of the C8 component of human complement.复发性脑膜炎球菌感染与人类补体C8成分的功能缺陷相关。
Clin Exp Immunol. 1980 Jan;39(1):53-9.
9
Immunological cross-reaction between a naturally occurring galactan, agarose, and an LPS locus for immune lysis of Neisseria meningitidis by human sera.一种天然存在的半乳聚糖、琼脂糖与人类血清对脑膜炎奈瑟菌进行免疫裂解的脂多糖位点之间的免疫交叉反应。
Clin Exp Immunol. 1981 Jan;43(1):20-7.
10
Granulocyte chemotaxis: an improved in vitro assay employing 51 Cr-labeled granulocytes.粒细胞趋化性:一种采用51铬标记粒细胞的改良体外测定法。
J Immunol. 1973 Jan;110(1):233-40.