Hereditary dysfunction of the third component of complement associated with a systemic lupus erythematosus-like syndrome and meningococcal meningitis.
1992 (English)In: Arthritis and Rheumatism, ISSN 0004-3591, E-ISSN 1529-0131, Vol. 35, no 5, 580-586 p.Article in journal (Refereed) Published
OBJECTIVE: We describe a dysfunction of C3 in a patient with a systemic lupus erythematosus (SLE)-like syndrome. Alternative pathway complement function was absent, but classical pathway complement function was partially intact.
METHODS: We used functional, preparative, and immunochemical techniques in the study.
RESULTS: The patient's C3 proved normally susceptible to trypsin proteolysis and partially resistant to classical pathway, but completely resistant to alternative pathway, convertase-dependent cleavage.
CONCLUSION: The dysfunction, thus, was caused by a failure of C3 to interact with the C3 convertases, rather than by a lack of a proteinase-sensitive cleavage site in the deficient protein.
Place, publisher, year, edition, pages
1992. Vol. 35, no 5, 580-586 p.
Medical and Health Sciences
IdentifiersURN: urn:nbn:se:uu:diva-265465PubMedID: 1575793OAI: oai:DiVA.org:uu-265465DiVA: diva2:865770