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Medientyp:
E-Artikel
Titel:
Factor H–mediated cell surface protection from complement is critical for the survival of PNH erythrocytes
Beteiligte:
Ferreira, Viviana P.;
Pangburn, Michael K.
Erschienen:
American Society of Hematology, 2007
Erschienen in:
Blood, 110 (2007) 6, Seite 2190-2192
Sprache:
Englisch
DOI:
10.1182/blood-2007-04-083170
ISSN:
0006-4971;
1528-0020
Entstehung:
Anmerkungen:
Beschreibung:
AbstractParoxysmal nocturnal hemoglobinuria (PNH) cells are partially (type II) or completely (type III) deficient in GPI-linked complement regulatory proteins CD59 and CD55. PNH III erythrocytes circulate 6 to 60 days in vivo. Why these cells are not lysed as rapidly by complement as unprotected foreign cells, which normally lyse within minutes, remains undetermined. Factor H plays a key role in the homeostasis of complement in fluid phase and on cell surfaces. We have recently shown that a recombinant protein encompassing the C-terminus of factor H (rH19-20) specifically blocks cell-surface complement regulatory functions of factor H without affecting fluid-phase control of complement. Here we show that PNH II and III cells become highly susceptible to complement-mediated lysis by nonacidified normal human serum in vitro, when the cell surface complement-regulatory functions of factor H are blocked. The results indicate that cells deficient in surface-bound regulators are protected for extended periods of time by factor H.