15th Page Flashcards

1
Q

What is the classical pathway?

A

specific immune system mechanism that triggers complement activation.

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2
Q

2nd oldest pathway

A

Classical pathway

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3
Q

main ab-directed mechanism for triggering complement activation

A

Classical Pathway

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4
Q

Which antibodies activate the classical pathway?

A

Only IgM and IgG (except IgG4) activate complement.

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5
Q

What are the three main stages of the classical pathway?

A
  1. Recognition unit
  2. Activation unit
  3. Membrane attack complex (MAC)
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6
Q

What components are involved in the recognition unit?

A

C1 (particularly C1q)

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7
Q

What does the activation unit consist of

A

C4, C2, C3

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8
Q

What does the membrane attack complex (MAC) consist of?

A

C5, C6, C7, C8, and C9.

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9
Q

1st MAC unit

A

C5

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10
Q

starts pore formation on membrane

A

C8

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11
Q

polymerizes to cause cell lysis

A

C9

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12
Q

binds to C5b in MAC

A

C6

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13
Q

binds to C5bC6 in MAC

A

C7

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14
Q

initiates membrane attack complex

A

C5

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15
Q

Key intermediate in all pathway

A

C3

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16
Q

Most concentrated complement component

A

C3

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17
Q

Binds to C4b form C3 convertase

A

C2

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18
Q

Part of the C3 convertase (C4b)

A

C4

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19
Q

Cleaves c4 and c2

A

C1s

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20
Q

Activate C1s

A

C1r

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21
Q

Binds to Fc of IgM and IgG

A

C1a

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22
Q

Alternative pathway

A

oldest complement activation pathway

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23
Q

Properdin system

A

Alternative pathway

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24
Q

Pillemer and his associates in the 1950s.

A

alternative pathway was described by:

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25
Q

It is antibody-independent

A

alternative pathway

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26
Q

unique serum proteins involved in the alternative pathway

A

Factor B and Factor D.

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27
Q

Which components are not used in the alternative pathway?

A

C1, C2, and C4

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28
Q

Key component of the alternative pathway?

A

C3, like in the classical pathway.

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29
Q

Conc. of Factor D in plasma?

A

lowest concentration in plasma at 1 µg/mL.

30
Q

What does Factor B (Bb) binds?

Alternative pathway

A

C3b to form C3 convertase.

31
Q

Factor D cleaves what?

Alternative pathway

A

Factor B.

32
Q

Properdin stabilizes what?

Alternative pathway

A

stabilizes C3 convertase and C5 convertase in the presence of Mg.

33
Q

What is the lectin (MBL) pathway?

A

3rd oldest complement activation pathway.

34
Q

What is a lectin?

A

A lectin is a protein that binds to carbohydrates.

35
Q

What does mannose-binding lectin (MBL) bind to?

A

mannose or related sugars such as N-acetylglucosamine to initiate the pathway.

36
Q

Where is MBL produced?

A

liver in acute-phase inflammatory reactions.

37
Q

What complex does MBL form?

A

complex with MASP-1 and MASP-2.

38
Q

What does MBL resembles?

A

C1a in both structure and function.

39
Q

What is the function of MASP-1?

A

help cleaves C4 and C2.

40
Q

What is the function of MASP-2?

A

cleaves C4 and C2.

41
Q

binds to mannose

A

MBL

42
Q

What is the function of C1 inhibitor (C1 iNH)?

A

Dissociates C1r and C1s from C1q.

43
Q

What does Factor I do?

A

Cleaves C3b and C4b.

Cofactor with I to inactivate C3b, prevents binding of B to C3b.

44
Q

Factor H

A

cofactor with I to inactivate C3b, prevents binding of B to C3b

45
Q

S protein (vitronectin)

A

Prevents attachment of the C5B67 complex to cell membrane.

46
Q

Factor H

A

The principal soluble regulator of the alternative pathway.

Dissociates C2b or Bb from binding sites, thus preventing formation of C3 convertase.

47
Q

cofactor with I to inactivate C4b

A

C4-binding protein

47
Q
A
48
Q

MIRL

Membrane Inhibitor of Reactive Lysis

A

Prevents insertion of C9 into cell membrane.

49
Q

Dissociates C2b or Bb from binding sites, thus preventing formation of C3 convertase

A

It binds C3b and C4b but has the greatest affinity for C3b.

50
Q

What happens once C3b and C4b are bound to CR1?

A

degraded by Factor I.

51
Q

cofactor with I

A

CR1

52
Q

Mediates transport of immune complexes, found in the peripheral blood cells

A

CR1

53
Q

Where is MCP (CD 46) found?

A

virtually all epithelial and endothelial cells except erythrocytes.

54
Q

MCP

A

most efficient cofactor for Factor I-mediated cleavage of C3b.

55
Q

How does MCP help control the alternative pathway?

A

Binding of Factor B to C3b is inhibited.

56
Q

complement deficiencies associated with lupus-like syndrome and recurrent infections?

A

C1 (q, r, or s), C2

Most common deficiency.

57
Q

What is the most severe complement deficiency?

A

C3

Associated with severe recurrent infections and glomerulonephritis (S. pyogenes).

58
Q

complement deficiency of glomerulonephritis (S. pyogenes)

A

C3

59
Q

severe recurrent infections

A

C3

60
Q

complement deficiency for atherosclerosis

A

C2

61
Q

most common complement deficiency

A

C2

62
Q

Which complement deficiencies are associated with Neisseria infections?

A

C5 - C8

Also associated with pneumococcal diseases.

63
Q

What is C1 INH deficiency associated with?

A

Hereditary angioedema

increased kinin-related proteins

may affect subcutaneous tissues, GI tract, respiratory

Causes swelling due to increased production of kinin-related proteins.

64
Q

complement deficiency of no known disease association

A

C9

64
Q

complement deficiency for neisseria infection

A

properdin

65
Q

complement deficiency for pneumococcal dse

A

C3, 2, 1, 4, properdin

66
Q

What is PNH?

A

Paroxysmal Nocturnal Hemoglobinuria

Associated with deficiency of complement regulators.

67
Q

What does the accumulation of fluids due to complement activation cause?

A

Edema or swelling

May affect subcutaneous tissues, GI tract, and respiratory tract.

68
Q

What is PIG-A?

A

Phosphatidyl Inositol Glycan - A

Will be constantly turned over to be activated until it becomes depleted.

69
Q

Recurrent pyogenic infection

A

Factor H & I

C3 will be constantly turn over to be activated until it depletes