C2 deficiency

Complement component 2 (C2) is a serine protease and is highly homologous to factor B. C2 is produced by hepatocytes, macrophages, and fibroblasts. C2 deficiency is an autosomal recesive disease and has been the most widely reported of all the components in this pathway. Deficient individuals have an increased incidence of systemic lupus erythematosus SLE and SLE-like syndromes, glomerulonephritis, vasculitis and pyogenic infections. Type I C2 deficiency is characterized by complete loss of the protein while type II C2 deficiency is characterized by a selective block in C2 secretion.

Alternative names


  • Defects of the classical complement cascade proteins


Autosomal recessive


+217000 Complement component 2 deficiency