Immunology

Immunodeficiency:
Defects of
Complement System
Defects of Complement System
• Abnormalities in
complement proteins
(Hypo-complementemia)
• Are genetic
abnormalities
• Inherited defects in the
synthesis of various
complement proteins
• Major defect in the
synthesis of C3 due to
defective C3 synthase
• Also defects in various
regulatory proteins like
Factor H & I
Defects of Complement System
• Majority of complement
deficiency is autosomal
recessive
• Properdin deficiency: X-
linked inheritance
• MBL deficiency can be
both
• Recurrent bacterial
infections particularly
Neisseria &
Streptococcus infections
• Also cause autoimmune
disorders like SLE,
vasculitis etc