Properdin system Factor

Pillemer er al, Science 120, 279 (1954). Combines with zymosan, q.v., the insol cell wall residue from yeast L. Pil -iemer, O. A. Ross, ibid. 121, 732 (1955). Participates also in a nonspecific manner in a variety of immunological reactions of normal serum. Isoln from human serum L. Pillemer et al, J. Exp. Med. 103, 1 (1956). Purification Spicer et al., U.S. pat. 3,030,038 (1962 to Merck Co.). Characterization of highly purified human properdin J. Pensky er al, J. Immunol 108, 142(1968). Activity studies O. Giitze, H. J. Muller-Eberhard, J. Exp Med. 139, 44 (1974). The human properdin system has been studied extensively it was the subject of long years of scientific controversy. Components of tha human properdin system that have been identified and characterized are propardin, factor B, [actor D and C3. 

B was originally characterized as one of the serum factors required for C3 destruction by the zymosan-activated properdin system. It was... 

Figure 6.4 The common genots pes of Factor B of the properdin system.

Complement deficiencies are associated with several diseases. Alternative pathway deficiencies are rare, but when they exist more than one-half of factor D or properdin-deficient individuals suffer from Neisseria infections of which 75% are fatal. Individuals with deficiencies in the MAC components, e.g., C5, C6, Cl, and C8, are also susceptible to infection with Neisseria. Deficiencies in C1, C4, and C2 are associated with systemic lupus erythematosus and glomerulonephritis. Hereditary angioedema, a disease characterized by recurrent submucosal and subcutaneous edema, is caused by a deficiency in Cl inhibitor. Complexes and interactions similar to those of the complement system are also characteristic of the clotting system (Chapter 36). 

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