Compement system 2

Lupus-like syndrome; recurrent infections

ASSOCIATED DISEASE:

C1

Lupus-like syndrome; recurrent infections, atherosclerosis

ASSOCIATED DISEASE:

C2

Severe recurrent infections, glomerulonephritis (S.pyogenes infection)

ASSOCIATED DISEASE:

C3

C3; Severe recurrent infections, glomerulonephritis (S.pyogenes infection)

most severe deficiency

Lupus like syndrome

ASSOCIATED DISEASE: C4

Neisseria infections

ASSOCIATED DISEASE: C5-C8

C9

Complement that has no known disease association

Hereditary angioedema

ASSOCIATED DISEASE: C1 INH

Paroxysmal nocturnal hemoglobinuria

ASSOCIATED DISEASE: DAF

Paroxysmal nocturnal hemoglobinuria

ASSOCIATED DISEASE: MIRL

C2; Lupus-like syndrome; recurrent infections, atherosclerosis

*most common deficiency

Recurrent pyogenic infections

ASSOCIATED DISEASE: FACTOR H or FACTOR I

Pneumococcal diseases, sepsis, Neisseria infections

MBL (disease ass)

Neisseria infections

Properdin (dse ass)

Pneumococcal disease

MASP-2 (dse ass)

Classical Pathway

It was the first complement pathway being studied, hence, named as classical

C1q, C1r, C1s

Recognition units of Classical pathway

C4,C2,C3

Activatiom units of Classical pathway

C5,6,7,8, and 9

Membrane attack complex(MAC) of Classical pathway

Properdin or Alternative pathway

It was the first complement pathway discovered

Lectin/ MBL pathway

The most recently described complement pathway

MASP-1 & MASP-2

Recognition unit of MBL

MBL

binds to mannose or related sugars in a calcium-dependent manner to initiate this pathway

Hemolytic uremic syndrome (HUS)

is the most common cause of renal failure in children and is characterized by hemolytic anemia, low platelet count, and acute renal failure

• CH5O

• AH50

2 types of hemolytic titration assay

hemolytic titration (CH50)

assay is most commonly used assay for classical pathway This assay measures the

amount of patient serum required to lyse 50% of a standardized concentration of antibody-sensitized sheep erythrocytes.