期刊
BLOOD
卷 110, 期 6, 页码 2190-2192出版社
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2007-04-083170
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资金
- NIDDK NIH HHS [R01 DK035081, R37 DK035081] Funding Source: Medline
- PHS HHS [DJ-35081] Funding Source: Medline
Paroxysmal nocturnal hemoglobinuria (PNH) cells are partially (type 11) or completely (type 111) 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 11 and III cells become highly susceptible to complement-mediated lysis by nonacidi-fied 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.
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