A high m.w. form of decay-accelerating factor (DAF-2) exhibits size abnormalities in paroxysmal nocturnal hemoglobinuria erythrocytes. 1987

T Kinoshita, and S I Rosenfeld, and V Nussenzweig

Decay-accelerating factor (DAF) is a 70,000 Mr membrane protein that inhibits the amplification of the complement cascade on cell surfaces. Monoclonal antibodies against different epitopes of the 70,000 Mr DAF (DAF-1) recognize a second band at the position of 140,000 Mr on a Western blot of total red cell ghost proteins or partially pure DAF subjected to electrophoresis under denaturing conditions. Like DAF-1, this polypeptide (DAF-2) has the ability to accelerate decay of the C3 convertase, C4b2a, and to reincorporate into red cell membranes. A population of erythrocytes from patients with paroxysmal nocturnal hemoglobinuria (PNH) lack DAF-1 and also DAF-2. In addition, in some patients' red cells bearing DAF-1 of normal Mr, DAF-2 is 5,000 to 10,000 Mr smaller than normal. The structural basis for these differences in size of DAF and its PNH variants is unknown.

UI MeSH Term Description Entries
D007163 Immunosorbent Techniques Techniques for removal by adsorption and subsequent elution of a specific antibody or antigen using an immunosorbent containing the homologous antigen or antibody. Immunoadsorbent Techniques,Immunoadsorbent Technics,Immunosorbent Technics,Immunoadsorbent Technic,Immunoadsorbent Technique,Immunosorbent Technic,Immunosorbent Technique,Technic, Immunoadsorbent,Technic, Immunosorbent,Technics, Immunoadsorbent,Technics, Immunosorbent,Technique, Immunoadsorbent,Technique, Immunosorbent,Techniques, Immunoadsorbent,Techniques, Immunosorbent
D008565 Membrane Proteins Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors. Cell Membrane Protein,Cell Membrane Proteins,Cell Surface Protein,Cell Surface Proteins,Integral Membrane Proteins,Membrane-Associated Protein,Surface Protein,Surface Proteins,Integral Membrane Protein,Membrane Protein,Membrane-Associated Proteins,Membrane Associated Protein,Membrane Associated Proteins,Membrane Protein, Cell,Membrane Protein, Integral,Membrane Proteins, Integral,Protein, Cell Membrane,Protein, Cell Surface,Protein, Integral Membrane,Protein, Membrane,Protein, Membrane-Associated,Protein, Surface,Proteins, Cell Membrane,Proteins, Cell Surface,Proteins, Integral Membrane,Proteins, Membrane,Proteins, Membrane-Associated,Proteins, Surface,Surface Protein, Cell
D008970 Molecular Weight The sum of the weight of all the atoms in a molecule. Molecular Weights,Weight, Molecular,Weights, Molecular
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D006457 Hemoglobinuria, Paroxysmal A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins. Paroxysmal Cold Hemoglobinuria,Paroxysmal Nocturnal Hemoglobinuria,Marchiafava-Micheli Syndrome,Paroxysmal Hemoglobinuria,Paroxysmal Hemoglobinuria, Cold,Paroxysmal Hemoglobinuria, Nocturnal,Cold Paroxysmal Hemoglobinuria,Hemoglobinuria, Cold Paroxysmal,Hemoglobinuria, Nocturnal Paroxysmal,Hemoglobinuria, Paroxysmal Cold,Hemoglobinuria, Paroxysmal Nocturnal,Marchiafava Micheli Syndrome,Nocturnal Paroxysmal Hemoglobinuria,Syndrome, Marchiafava-Micheli
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000911 Antibodies, Monoclonal Antibodies produced by a single clone of cells. Monoclonal Antibodies,Monoclonal Antibody,Antibody, Monoclonal
D018958 CD55 Antigens GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex. Antigens, CD55,Decay-Accelerating Factor,CD55 Antigen,Complement Decay-Accelerating Factor,Complement Decay Accelerating Factor,Decay Accelerating Factor,Decay-Accelerating Factor, Complement

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