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.
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.
Authors: Georgia Sfyroera; Daniel Ricklin; Edimara S Reis; Hui Chen; Emilia L Wu; Yiannis N Kaznessis; Kristina N Ekdahl; Bo Nilsson; John D Lambris Journal: J Immunol Date: 2015-02-23 Impact factor: 5.422
Authors: Anna E Engberg; Kerstin Sandholm; Fredrik Bexborn; Jenny Persson; Bo Nilsson; Gunnar Lindahl; Kristina N Ekdahl Journal: Biomaterials Date: 2009-01-25 Impact factor: 12.479