A pedigree of deficiency of the ninth component of complement (C9)

Kusaba, Tomohiro; Kisu, Tatsuro; Inaba, Shinji; Sakai, Kotaro; Okochi, Kazuo; Yanase, Toshiyuki

doi:10.1007/bf01876786

Cited by 4 publications

(2 citation statements)

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“…A limitation of the attempts to prevent meningococcal infection in complement-deficient individuals by immunoprophylaxis is the absence of group B capsular polysaccharide in the meningococcal vaccine and the failure of humans to generate a significant humoral response to this sialic acid homopolymer. Despite the relatively greater frequency of meningococcal infection caused by unusual serogroups in complement-deficient compared with normal individuals (116,203), group B meningococcal disease still accounts for a significant proportion of meningococcal infections in deficient persons (Table 6) (303). Consequently, Potter et al (279) used monthly injections of benzathine penicillin G as prophylaxis for recurrent meningococcal disease.…”

Section: Prevention Of Infection In Complement-deficient Individualsmentioning

confidence: 99%

Infectious diseases associated with complement deficiencies.

Figueroa

Densen

1991

Clinical Microbiology Reviews

144

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tem were first described between 1888 and 1894 (300). First, nonimmune serum that by itself had no bactericidal activity. Nuttall discovered that sheep blood had mild bacterial Thus, the bactericidal activity of immune serum was depenactivity for Bacillus anthracis that was lost when the blood dent on both alexin and a heat-stable component. was heated to 55°C. Next, Buchner demonstrated that the At the turn of the century, Ehrlich proposed a model for bactericidal activity of fresh serum was due to a heat-labile serum immunity involving these two fractions. The heatfactor that he termed alexin. In 1894, Pfeiffer found that, stable serum fraction, amboceptor or antibody, contained when blood from guinea pigs that had survived cholera was two binding sites; one bound to the organism and the other mixed with the vibrio and injected into naive guinea pigs, it bound to the heat-labile factor, complement. Thus, specificity of bactericidal activity depended on antibody, not complement. Several investigators later discovered that the * Corresponding author. complement fraction consisted of more than one component.

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Section: Prevention Of Infection In Complement-deficient Individualsmentioning

confidence: 99%

Infectious diseases associated with complement deficiencies.

Figueroa

Densen

1991

Clinical Microbiology Reviews

144

View full text Add to dashboard Cite

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Section: Prevention Of Infection In Complement-deficient Individualsmentioning

confidence: 99%

Infectious diseases associated with complement deficiencies

1991

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The complement system consists of both plasma and membrane proteins. The former influence the inflammatory response, immune modulation, and host defense. The latter are complement receptors, which mediate the cellular effects of complement activation, and regulatory proteins, which protect host cells from complement-mediated injury. Complement activation occurs via either the classical or the alternative pathway, which converge at the level of C3 and share a sequence of terminal components. Four aspects of the complement cascade are critical to its function and regulation: (i) activation of the classical pathway, (ii) activation of the alternative pathway, (iii) C3 convertase formation and C3 deposition, and (iv) membrane attack complex assembly and insertion. In general, mechanisms evolved by pathogenic microbes to resist the effects of complement are targeted to these four steps. Because individual complement proteins subserve unique functional activities and are activated in a sequential manner, complement deficiency states are associated with predictable defects in complement-dependent functions. These deficiency states can be grouped by which of the above four mechanisms they disrupt. They are distinguished by unique epidemiologic, clinical, and microbiologic features and are most prevalent in patients with certain rheumatologic and infectious diseases. Ethnic background and the incidence of infection are important cofactors determining this prevalence. Although complement undoubtedly plays a role in host defense against many microbial pathogens, it appears most important in protection against encapsulated bacteria, especially Neisseria meningitidis but also Streptococcus pneumoniae, Haemophilus influenzae, and, to a lesser extent, Neisseria gonorrhoeae. The availability of effective polysaccharide vaccines and antibiotics provides an immunologic and chemotherapeutic rationale for preventing and treating infection in patients with these deficiencies.

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