Inhibiting complement activation on cells at the step of C3 cleavage.
Nearly half of the proteins in the complement system serve in regulation. Control at the central step of C3 activation is provided by an orchestrated interplay of membrane and plasma regulators. A model system employing Chinese hamster ovary (CHO) cells transfected with human regulators was employed to assist in making functional comparisons. Also, in this experimental setup, the pathway and magnitude of complement activation can be varied while monitoring C4b/C3b deposition and cleavage as well as cytotoxicity. This review describes lessons learned and the application of this model for functionally characterizing mutations in regulators associated with atypical hemolytic uremic syndrome.
Liszewski
M Kathryn
MK
Washington University School of Medicine, Department of Internal Medicine, Division of Rheumatology, 660 S. Euclid, Box 8045, St. Louis, MO 63110, USA.
Fang
Celia J
CJ
Atkinson
John P
JP
D000818:Animals; D018958:CD55 Antigens / Q000502:physiology; D016466:CHO Cells; D003167:Complement Activation; D003176:Complement C3 / Q000378:metabolism*; D006224:Cricetinae; D003412:Cricetulus; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000235:genetics; D006801:Humans; D051916:Membrane Cofactor Protein / Q000235:genetics / Q000502:physiology; D009154:Mutation
D018958:CD55 Antigens; D003176:Complement C3; D051916:Membrane Cofactor Protein