Complete deficiency of 20 KDa homologous restriction factor (HRF20) and restoration with purified HRF20 |
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Authors: | N Okada R Harada R Taguchi H Okada |
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Institution: | Department of Microbiology, Fukuoka University School of Medicine, Japan. |
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Abstract: | 20 KDa homologous restriction factor (HRF20) is a membrane glycoprotein which inhibits formation of membrane attack complexes of homologous complement. Erythrocytes from a patient who is completely deficient in HRF20 were readily hemolyzed by homologous complement activated by sucrose or by acidification as in paroxysmal nocturnal hemoglobinuria (PNH). After incubating PNH erythrocytes (PNH-E) with purified HRF20, the cells were analyzed by flow cytometry using a monoclonal antibody to HRF20 and shown to have the antigen absorbed. These PNH-E acquired resistance to hemolysis by homologous complement suggesting that HRF20 may be successfully used for treatment of these patients. |
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