Complete deficiency of 20 KDa homologous restriction factor (HRF20) and restoration with purified HRF20.
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.
Okada
N
N
Department of Microbiology, Fukuoka University School of Medicine, Japan.
Harada
R
R
Taguchi
R
R
Okada
H
H
D000328:Adult; D001798:Blood Proteins; D018957:CD59 Antigens; D002352:Carrier Proteins; D004912:Erythrocytes / Q000378:metabolism*; D005434:Flow Cytometry; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood; D006461:Hemolysis; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000172:deficiency*; D008970:Molecular Weight
D001798:Blood Proteins; D018957:CD59 Antigens; D002352:Carrier Proteins; D008562:Membrane Glycoproteins; C051590:CD59 protein, human