Properdin deficiency

Individuals with asplenia, complement deficiency, or properdin deficiency... 

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). 

Bathum L, Hansen H, Teisner B et al 2006 Association between combined properdin and mannose-binding lectin deficiency and infection with Neisseria meningitidis. Mol Immunol 43 473-479... 

Because of the total obstruction in the activation of the classical complement pathway in their serum, C4 deficient guinea pig serum has played an important role in elucidating the function of the alternative or properdin pathway ". ... 

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