TY - JOUR
T1 - Inhibiting complement activation on cells at the step of C3 cleavage
AU - Liszewski, M. Kathryn
AU - Fang, Celia J.
AU - Atkinson, John P.
PY - 2008/12/30
Y1 - 2008/12/30
N2 - 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.
AB - 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.
KW - CD46)
KW - Complement regulation
KW - Hemolytic uremic syndrome
KW - Membrane cofactor protein (MCP
UR - http://www.scopus.com/inward/record.url?scp=57649143877&partnerID=8YFLogxK
U2 - 10.1016/j.vaccine.2008.11.001
DO - 10.1016/j.vaccine.2008.11.001
M3 - Article
C2 - 19388160
AN - SCOPUS:57649143877
SN - 0264-410X
VL - 26
SP - I22-I27
JO - Vaccine
JF - Vaccine
IS - SUPPL. 8
ER -