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Clin Exp Immunol. 1970 August; 7(2): 133–137. | PMCID: PMC1712843 |
Sub-units of the first complement component in immunologic deficiency syndromes: independence of C1s and C1q R. M. Stroud, K. Nagaki, R. J. Pickering, H. Gewurz, R. A. Good, and M. D. Cooper Abstract Normal concentrations of C1 esterase, C1s, have been found in nineteen individuals with a variety of immunologic deficiency syndromes. C1q levels are markedly low in patients with lymphopenic hypogammaglobulinaemia. The lack of correlation of serum concentrations of C1s with C1q in several patients suggests a separate mode of synthesis or catabolism for these C1 subunits. Furthermore, the extreme C1q deficiency in lymphopenic hypogammaglobulinaemic patients may play a role in their serious prognosis; however the normal C1s concentrations would allow some complement mediated functions. Full text Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (564K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References. These references are in PubMed. This may not be the complete list of references from this article. - Colten HR, Gordon JM, Borsos T, Rapp HJ. Synthesis of the first component of human complement in vitro. J Exp Med. 1968 Oct 1;128(4):595–604. [PubMed]
- Cooper MD, Gabrielsen AE, Good RA. Role of the thymus and other central lymphoid tissues in immunological disease. Annu Rev Med. 1967;18:113–138. [PubMed]
- Dias Da Silva W, Lepow IH. Complement as a mediator of inflammation. II. Biological properties of anaphylatoxin prepared with purified components of human complement. J Exp Med. 1967 May 1;125(5):921–946. [PubMed]
- Gewurz H, Pickering RJ, Christian CL, Snyderman R, Mergenhagen SE, Good RA. Decreased C'-1q protein concentration and agglutinating activity in agammaglobulinaemia syndromes: an inborn error reflected in the complement system. Clin Exp Immunol. 1968 Jun;3(5):437–445. [PubMed]
- Gewurz H, Pickering RJ, Naff G, Good RA. Decreased properdin activity in Swiss-type lymphopenic agammaglobulinemia. Lancet. 1968 May 4;1(7549):982. [PubMed]
- Hanauer LB, Christian CL. Clinical studies of hemolytic complement and the 11S component. Am J Med. 1967 Jun;42(6):882–890. [PubMed]
- JONSEN J, KASS E. Agammaglobulinemia; report of titrations of complement components in a case. Acta Pathol Microbiol Scand. 1957;41(5):393–396. [PubMed]
- Kohler PF, Müller-Eberhard HJ. Immunochemical quantitation of the third, fourth and fifth components of human complement: concentrations in the serum of healthy adults. J Immunol. 1967 Dec;99(6):1211–1216. [PubMed]
- Kohler PF, Müller-Eberhard HJ. Complement-immunoglobulin relation: deficiency of C'1q associated with impaired immunoglobulin G synthesis. Science. 1969 Jan 31;163(866):474–475. [PubMed]
- LEPOW IH, NAFF GB, TODD EW, PENSKY J, HINZ CF. Chromatographic resolution of the first component of human complement into three activities. J Exp Med. 1963 Jun 1;117:983–1008. [PubMed]
- Mancini G, Carbonara AO, Heremans JF. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. [PubMed]
- MULLER-EBERHARD HJ, KUNKEL HG. Isolation of a thermolabile serum protein which precipitates gamma-globulin aggregates and participates in immune hemolysis. Proc Soc Exp Biol Med. 1961 Feb;106:291–295. [PubMed]
- MUELLER-EBERHARD HJ, LEPOW IH. C'1 ESTERASE EFFECT ON ACTIVITY AND PHYSICOCHEMICAL PROPERTIES OF THE FOURTH COMPONENT OF COMPLEMENT. J Exp Med. 1965 May 1;121:819–833. [PubMed]
- Müller-Eberhard HJ, Polley MJ, Calcott MA. Formation and functional significance of a molecular complex derived from the second and the fourth component of human complement. J Exp Med. 1967 Feb 1;125(2):359–380. [PubMed]
- Nagaki K, Stroud RM. The relationship of the hemolytic activity of active C'1s to its TAMe esterase action: a new method of purification and assay. J Immunol. 1969 Feb;102(2):421–430. [PubMed]
- O'Connell EJ, Enriquez P, Linman JW, Oleich GJ, McDuffie FC. Absence of activity of first component of complement in man: association with thymic alymphoplasia and defective inflammatory response. J Lab Clin Med. 1967 Nov;70(5):715–724. [PubMed]
- Stroud RM, Austen KF, Mayer MM. Catalysis of C'2 fixation by C'la. Reaction kinetics, competitive inhibition by TAMe, and transferase hypothesis of the enzymatic action of C'la on C'2, one of its natural substrates. Immunochemistry. 1965 Sep;2(3):219–234. [PubMed]
- Tomasi TB, Jr, Bienenstock J. Secretory immunoglobulins. Adv Immunol. 1968;9:1–96. [PubMed]
- WARD PA, COCHRANE CG, MUELLER-EBERHARD HJ. THE ROLE OF SERUM COMPLEMENT IN CHEMOTAXIS OF LEUKOCYTES IN VITRO. J Exp Med. 1965 Aug 1;122:327–346. [PubMed]
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