Complement. Introduction The complement system is one of the major effector mechanisms of humoral immunity Is an important effector mechanism of innate.

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Presentation transcript:

Complement

Introduction The complement system is one of the major effector mechanisms of humoral immunity Is an important effector mechanism of innate immunity. Jules Bordet 1919 The complement system consists of serum and cell surface proteins Activation of complement involves the sequential proteolysis zymogenszymogens

Complement nomenclature C 3 C3aC3a Convertase C3bC3b

The classical pathway of complement activation

The classical pathway

C1q molecule

The classical pathway of complement activation

The alternative pathway

TickoverTickover

The alternative-pathway C3 and C5 convertases are both stabilized by properdin (factor P)

Lectin Pathway

Lectin Pathway of Complement Activation Ficolin

MAC formation

late steps of complement activation and formation of the MAC

Regulator of complement activation

Inhibition of the formation of C3 convertases

Factor I-mediated cleavage of C3b Regulator of complement activation

Regulation of formation of the MAC

The Roles

The Good

Functions of complement

Receptors for Fragments of C3

Inflammatory effects of complement

The bad

C3 deficiency Sever recurrent infection with encapsulated bacteria Begain shortly after birth

MAC (C5-C9) deficiency Susceptible to N. meningitidis infection

Complement deficiency

The ugly

C1q, C2 and C4 deficiency Associated with the development of SLE.

C1INH Deficiency

PNH Deficiency in DAF or CD 59

fetal loss in antiphospholipid syndrome.

Examples of pathological conditions involving the complement system.

Clinical effects of complement deficiency

The End