Relationship between the membrane inhibitor of reactive lysis and the erythrocyte phenotypes of paroxysmal nocturnal hemoglobinuria.

The Journal of Clinical Investigation
M H HolguinC J Parker

Abstract

Susceptibility to hemolysis initiated by activated cobra venom factor (CoF) complexes is a characteristic that distinguishes the most complement-sensitive type III erythrocytes of paroxysmal nocturnal hemoglobinuria (PNH) from the intermediately sensitive type II and the normally sensitive type I cells. Recently we isolated a membrane constituent from normal erythrocytes that inhibits CoFBb-initiated hemolysis, and this protein was designated membrane inhibitor of reactive lysis (MIRL). To investigate the molecular basis of the variability in complement sensitivity among PNH erythrocytes, the surface expression of MIRL and decay accelerating factor (DAF) on the three phenotypes of PNH was quantified immunochemically. Both complement regulatory proteins were markedly deficient on the erythrocytes from a patient with predominately type III cells. The erythrocytes from patients with a majority of either type II or I cells were also significantly deficient in both MIRL and DAF. While cytofluorometric analysis confirmed the quantitative deficiencies, segregation of erythrocytes into discrete subpopulations that expressed either no MIRL or normal amounts of MIRL was not observed. The results of immunoprecipitation studies were consis...Continue Reading

References

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Citations

Mar 12, 2003·International Journal of Hematology·Wendell F Rosse, Junichi Nishimura
Mar 12, 2003·International Journal of Hematology·Gabrielle Meyers, Charles J Parker
Mar 29, 2011·Archivum Immunologiae Et Therapiae Experimentalis·Jacek NowakBarbara Zupańska
Nov 1, 1996·Critical Reviews in Oncology/hematology·H Nakakuma, T Kawaguchi
Jan 1, 1995·Transfusion clinique et biologique : journal de la Société française de transfusion sanguine·M J Telen
Feb 1, 1995·Immunology and Cell Biology·F F YuanA Fletcher
Mar 29, 2002·British Journal of Haematology·Charles J Parker
Oct 3, 1991·The New England Journal of Medicine·R E WareW F Rosse
May 1, 1991·Proceedings of the National Academy of Sciences of the United States of America·S HiroseM E Medof
Feb 27, 2001·Current Opinion in Hematology·W F Rosse
Oct 1, 1990·British Journal of Haematology
Jun 1, 1994·The Journal of Clinical Investigation·E T Yeh, W F Rosse
Dec 17, 2008·Hematology·Charles J Parker
Jul 30, 2005·Blood·Charles ParkerUNKNOWN International PNH Interest Group
Jun 7, 2008·Blood·Régis Peffault de LatourUNKNOWN French Association of Young Hematologists
Sep 7, 2007·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·N Jewel SamadderRodrigo Cavalcanti
Dec 24, 2013·Leukemia & Lymphoma·Aziz NazhaSrdan Verstovsek
Mar 31, 2009·Hematology/oncology Clinics of North America·Charles J Parker
Dec 1, 1991·Baillière's Clinical Haematology·M J Telen, W F Rosse
Mar 24, 2007·Experimental Hematology·Charles J Parker
Sep 7, 2013·Thrombosis and Haemostasis·A Verschoor, H F Langer
Jan 1, 1995·Transfusion Medicine Reviews·M J Telen
Feb 20, 2004·Hematology·Tsutomu Shichishima, Hideyoshi Noji

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