Regal Part I

Question 1

What complement deficiencies cause SLE?

Answer 1

C1q, C1r, C1s, C4, C2, C1-INH, C3

Question 2

What complement deficiencies cause Glomeronephritis?

Answer 2

C1q, C1r, C1s, C4, C2, C3

Question 3

What two pathways help get rid of Neisserria most?

Answer 3

Terminal lytic pathway & Alternate Pathway, C3

Question 4

What pathway deficiencies are associated with immune complex or autoimmune disease?

Answer 4

Classical pathway & C3 deficiencies

Question 5

What types of infections are associated with MBL deficiency?

Answer 5

Serious pyrogenic infections

Question 6

Deficiencies associated with what pathways are associated with pyrogenic infections and infection with encapsulated bacteria?

Answer 6

Classical & Alternative

Question 7

What are the three different start signals for the three complement pathways?

Answer 7

1. Classical - Antigen antibody complexes
2. Mannose binding lectin - mannose
3. Alternative - LPS, carbohydrates, etc.

Question 8

What covalently binds in the classical pathway?

Answer 8

C3b and C4b covalently bind via thioester bonds!

Question 9

What are key points to know about the classical pathway?

Answer 9

• Activation in conjunction with specific antibody
• C3b and C4b covalently bind via thioester bonds
• Enzymatic cleavage of proteins with amplification

Question 10

What does the MBL pathway recognize?

Answer 10

Polysaccharides/sugars on microbes!

Question 11

What kinds of polysaccharide microbes trigger the MBL Pathway?

Answer 11

Salmonella, Listeria, Neiserria, Candida

Question 12

What is the significance of activator surfaces in the alternative pathway?

Answer 12

Activator surface - deficient in sialic acid
Non-activator surface - has lots of sialic acid
In general, bacteria are deficient in sialic acid. This creates a good activator surface for complement.

Question 13

What is the alternative pathway C3 Convertase?

Answer 13

C3bBb

Question 14

What is the classical pathway C3 Convertase?

Answer 14

C4b2a

Question 15

What is C1 Inh (Inhibitor) Deficiency?

Answer 15

• HAE (Hereditary angioedema)
• Recurrent episodes of localize edema in skin, GI tract or larynx
• C1 inhibitor inhibits C1 esterase
• Uncontrolled complement activation leads to consumption of C4 and C2 (possibility of immune complex disease as well)

Question 16

C3 is cleaved by both. . .

Answer 16

. . .the alternative and classical pathway C4 convertase

Question 17

C3b and C4b. . .

Answer 17

covalently attach to the activator surface