Analysis of PI (phosphatidylinositol)-anchoring antigens in a patient of paroxysmal nocturnal hemoglobinuria (PNH) reveals deficiency of 1F5 antigen (CD59), a new complement-regulatory factor.
FACS analysis together with PIPLC treatment was applied to PI-anchoring antigens such as DAF (decay-accerelating factor, CD55), 1F5 antigen (CD59), CD14 and CD16 on the cell surfaces of blood cells from a normal adult and a male patient with paroxysmal nocturnal hemoglubinuria (PNH). Through the extensive analysis, this patient proved to be completely defective in 1F5 antigen, a newly found complement-regulatory protein, on all the blood cells tested. In normal blood cells such as lymphocytes, monocytes and granulocytes, 1F5 antigen was expressed as one of PI-anchoring proteins. In contrast to most of PNH patients, this patient reserved DAF, CD14 and CD16 at normal levels in his erythrocytes, monocytes and granulocytes. Also, there were no significant differences between the normal adult and the patient in the activities of erythrocyte acetylcholinesterase and granulocyte alkaline phosphatase which were also known to be PI-anchoring enzymes. Thus, deficiency of 1F5 antigen must be deeply related to the clinical symptoms of PNH in this patient.
Faculty of Pharmaceutical Sciences, Nagoya City University, Japan.
R
R
Taguchi
Y
Y
Funahashi
H
H
Ikezawa
I
I
Nakashima
D000328:Adult; D000943:Antigens, Differentiation / Q000032:analysis / Q000172:deficiency*; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D018958:CD55 Antigens; D018957:CD59 Antigens; D003169:Complement Inactivator Proteins; D004912:Erythrocytes / Q000276:immunology; D005434:Flow Cytometry; D006098:Granulocytes / Q000276:immunology; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology*; D006801:Humans; D018950:Lipopolysaccharide Receptors; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D008565:Membrane Proteins / Q000032:analysis; D009000:Monocytes / Q000276:immunology; D043265:Phosphatidylinositol Diacylglycerol-Lyase; D010716:Phosphatidylinositols / Q000276:immunology*; D010727:Phosphoric Diester Hydrolases / Q000494:pharmacology; D011961:Receptors, Fc / Q000032:analysis; D017452:Receptors, IgG
D000943:Antigens, Differentiation; D015214:Antigens, Differentiation, Myelomonocytic; D018958:CD55 Antigens; D018957:CD59 Antigens; D003169:Complement Inactivator Proteins; D018950:Lipopolysaccharide Receptors; D008565:Membrane Proteins; D010716:Phosphatidylinositols; D011961:Receptors, Fc; D017452:Receptors, IgG; D010727:Phosphoric Diester Hydrolases; D043265:Phosphatidylinositol Diacylglycerol-Lyase