Complement

Question 1

C1 -INH Function and which pathway is involved

Answer 1

Function: C1r dissociates from C1 complex, and MASP-2 dissociates from MBL-MASP complex

Pathways: Classical and Lectin

Question 2

Factor I: Function and which pathway is affected

Answer 2

Function: Inactivates C3b and C4b

Pathways: all three (I think)

Question 3

Factor H: Function and which pathway is affected

Answer 3

Function: Cofactor with I to inactivate C3b; specifically prevents binding B to C3b

Pathways: alternative

Question 4

C4BP (C4 binding protein)

Answer 4

Function: acts as a cofactor with I to inactivate C4b

Pathways: Classical, Lectin

Question 5

S protein (vitronectin)

Answer 5

Function: prevents attachment of C5b67 complex to cell membrane

Pathways: all 3

Question 6

Most control proteins are aimed at halting accumulation of C3b

Answer 6

Most control proteins are aimed at halting accumulation of C3b

Question 7

Receptor DAF (CD55); what is the ligand, and the function

Answer 7

Ligand: C3b, C4b

Function: prevents formation of C3 convertase (the proteins dissociate from each other)

Question 8

Receptor MIRL (CD59); what is the ligand, and the function

Answer 8

Ligand: C8

Function: prevents insertion of C9 into cell membrane of invader

Question 9

Receptor MCP (CD46); what is the ligand, and the function

Answer 9

Ligand: C3b, C4b

Function: cofactor for factor I (so helps in cleaving C3b and C4b)

Question 10

deficiency in C1 (q, r, or s) is associated with what disease?

Answer 10

Lupus-like syndrome; recurrent infections

Question 11

deficiency in C2 is associated with what disease?

Answer 11

Lupus-like syndrome; recurrent infections; atherosclerosis (buildup of fat in artery walls)

Question 12

deficiency in C3 is associated with what disease?

Answer 12

recurrent infections: glomerulonephritis (damage to tiny filters inside kidneys)

Question 13

deficiency in C4 is associated with what disease?

Answer 13

Lupus like syndrome

Question 14

deficiency in C5-8 is associated with what disease?

Answer 14

Neisseria infections

Question 15

deficiency in C9 is associated with what disease?

Answer 15

No known disease association

Question 16

deficiency in C1-INH is associated with what disease?

Answer 16

Hereditary angioedema (similar to hives, w/o pain)

Question 17

deficiency in DAF is associated with what disease?

Answer 17

Paroxysmal nocturnal hemoglobinuria (disease of the blood)

Question 18

deficiency in MIRL is associated with what disease?

Answer 18

Paroxysmal nocturnal hemoglobinuria (disease of the blood)

Question 19

deficiency in Factor H or I is associated with what disease?

Answer 19

Recurrent pyogenic infections

Question 20

deficiency in MBL is associated with what disease?

Answer 20

Pneumococcal disease, sepsis, Neisseria infections

Question 21

deficiency in Properdin is associated with what disease?

Answer 21

Neisseria infections

Question 22

deficiency in MASP-2 is associated with what disease?

Answer 22

Pneumococcal disease