Homozygous C2 Deficiency: Association with Defective Alternative Pathway Function and Immunoglobulin Deficiency

International Archives of Allergy and Immunology

Volumn 110, Issue 2, 1996, Pages 195-198

  Sanal, Özden ^a   Yel, Leman ^a   Tezcan, Ilhan ^a   Ersoy, Fügen ^a   Berkel, Izzet ^a  

^a HACETTEPE UNIVERSITY   (Turkey)

Author keywords

Alternative pathway; C2 deficiency; Complement; IgG subclass deficiency

Indexed keywords

COMPLEMENT COMPONENT C2; IMMUNOGLOBULIN A; IMMUNOGLOBULIN G1; IMMUNOGLOBULIN G2;

ADOLESCENT; ADULT; ARTICLE; CHILD; CLINICAL ARTICLE; COMPLEMENT ALTERNATIVE PATHWAY; COMPLEMENT DEFICIENCY; FAMILY STUDY; FEMALE; HEMOLYSIS; HUMAN; IMMUNOGLOBULIN A DEFICIENCY; INFECTION SENSITIVITY; MALE; PRIORITY JOURNAL;

EID: 0030007069     PISSN: 10182438     EISSN: 14230097     Source Type: Journal    
DOI: 10.1159/000237287     Document Type: Article

References (21)

1. Agncllo V: Complement deficiency states. Mcdieine 1978;57:1-23.
2. Morgan BP, Walport MJ: Complement deficiency and disease. Immunol Today 1991:12: 301-311.
3. Coltcn HR: Complement deficiencies. Annu Rev Immunol 1992;10:809-834.
4. Glass D, Raum D, Gibson I, Stillman JS, Schur PH: Inherited deficiency of the second component of complement. Rheumatic disease associations. J Clin Invest 1976;58:853-861.
5. Figueroa JE. Densen P: Infectious diseases associated with complement deficiencies. Clin Microbiol Rev 1991;4:359-395.
6. Thong YII, Simpson DA, Miiller-Eberhard HJ: Homozygous deficiency of the second component of complement presenting with recurrent bacterial meningitis. Arch Dis Child 1980;55: 471-473.
7. Hyatt AC, Altenburger KM, Johnston RB. Win-kelstein JA: Increased susceptibility to severe pyogenic infections in patients with an inherited deficiency of the second component of complement. J Pediatr 1981;98:417-419.
8. Sampson HA, Walchner AM, Baker PJ: Recurrent pyogenic infections in individuals with absence of the second component of complement. J Clin Immunol 1982;2:39-45.
9. Leggiadro RJ, Warren AB, Swift AJ, Winkel-stein JA: Meningococcemia in genetically determined deficiency of the second component of complement. J Infect Dis 1983:148:941.
10. Johnson CA, Densen P, Wetsel RA, Cole FS, Goekcn NE. Colten HR: Molecular heterogeneity ofC2 deficiency. N Engl J Med 1992;326: 871-874.
11. Mayer MM: Complement and complement fixation; in Kabat EA, Mayer MM (eds): Experimental lmmunochemistry, ed 2. Springfield. Thomas, 1961. pp 149-153.
12. Joiner KA, Hawiger A, Gelfand JA: A study of optimal reaction conditions for an assay of the human alternative complement pathway. Am J Clin Pathol 1983;79:65-72.
13. Sanal O, Loos M, Ersoy F, Kanra G, Secmcer G, Tezcan I: Complement component deficiencies and infection: C5 and C3 deficiencies in three families. EurJ Pediatr 1992;151:676-679.
14. Newman SL, Vogler LB, Feigin RD, Johnston RB: Recurrent septicemia associated with congenital deficiency of C2 and partial deficiency of factor B and the alternative complement pathway. N Engl J Med 1978;299:290-292.
15. Schwertz R, Esser E, Seger RA, Rubinstein A, Hauptmann G. Wahn V: Defective activation of the alternative pathway of complement in patients with homozygous C2 deficiency: Studies in two unrelated families. Eur J Pediatr 1991; 150:647-651.
16. Johnston RB Jr, Klemperer MR, Alper CA, Rosen FS: The enhancement of bacterial phagocytosis by scrum: The role of complement components and two cofactors. J Exp Med 1969; 129: 1275-1290.
17. Winkclstcin JA, Shin HS, Wood WB Jr.: Heat labile opsonins to pneumococcus. III. The participation of immunoglobulin and of the alternate pathways of C3 activation. J Immunol 1972;108:1681-1689.
18. Gallin II, Clark RA, Frank MM: Kinetic analysis of chemotactic factor generation in human serun via activation of the classical and alternate complement pathways. Clin Immunol Im-munopathol 1975;3:334-346.
19. Ishikawa N, Nonaka M, Wetsel RA, Colten HR: Murine complement C2 and factor B genomic and cDNA cloning reveals different mechanisms for multiple transcripts of C2 and B. J Biol C'hem 1990;265:19040-19046.
20. Klemperer MR, Woodwarth HC, Rosen FS, Austen KF: Hereditary deficiency of the second component of complement (C'2) in man. J Clin Invest 1966;45:880-890.
21. Awdeh ZL, Raum DD, Glass D, Agnello V, Schur PH, Johnston RB Jr., Gclfand EW, Ballow M, Yunis E, Alper CA: Complement-human histocompatibility antigen haplotypes in C2 deficiency. J Clin Invest 1981;67:581-583.