2S [LEC]: Complement Flashcards

1
Q

An integral part in mediating immune response and bridges between the innate and adaptive immunity

A

Complement

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2
Q

Where is the C1 produced?

A

Intestinal epithelial cells

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3
Q

Where is the factor D produced?

A

Adipose tissue

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4
Q

Cells that produce properdin and C7

A

Immune cells (monocytes, macrophages, neutrophils)

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5
Q

Complex series of more than 30 circulating and membrane-bound proteins

A

Complement

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6
Q

Part of C1 that binds to the Fc fragment of the antibody

A

C1q

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7
Q

Most abundant C protein in serum that is considered as the convergence point of all pathways and marker of inflammation

A

C3

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8
Q

2nd most abundant C protein in the serum

A

C4

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9
Q

C protein fragment that is considered a very potent opsonin

A

C3b

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10
Q

A membrane-bound glycoprotein that binds to C3b and C4b, playing a crucial role in immune complex clearance and regulating complement activation

A

CR1/ CD35

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11
Q

A C protein fragment that can act as a kinin activator

A

C2b

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12
Q

C protein fragments that can act as chemotaxins

A

C5a, C5b, C6, C7

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13
Q

C protein fragments that can act as anaphylatoxins

A

C3a, C4a, C5a

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14
Q

Most potent C protein fragment anaphylatoxin

A

C5a

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15
Q

C protein fragment/s that is/are responsible in the neutralization of viruses

A

C4b, C1

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16
Q

In the alternative pathway, this C protein fragment is recognized by CR2 present in B cells to enhance its response to microbe

A

C3d

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17
Q

C protein fragment that releases neutrophils from the bone marrow

A

C3c

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18
Q

C protein fragment that releases hydrolytic enzymes from neutrophils

A

C5a

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19
Q

This scientist introduced the role of complementing antibodies in destroying microorganisms

A

Ehrlich

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20
Q

This scientist elucidated the role of C’ in the immune system

A

Bordet

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21
Q

T/F: All the three pathways have the same components of the MAC

A

True

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22
Q

Other than antibodies, which factors can activate the classical pathway?

A

CRP, Viruses, Bacteria (Mycoplasma)

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23
Q

Specific part of the Fc region where the C1q binds if the antibody is an IgG

A

CH2

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24
Q

Specific part of the Fc region where the C1q binds if the antibody is an IgM

A

CH3

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25
Q

Parts of the C1 that act as zymogens to generate enzyme activity

A

C1r, C1s

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26
Q

Most effective IgG in activating complement

A

IgG3

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27
Q

Least effective IgG in activating complement

A

IgG2

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28
Q

First complement pathway to act

A

Alternative pathway

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29
Q

Second complement pathway to act

A

Lectin pathway

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30
Q

Last complement pathway to act

A

Classical pathway

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31
Q

The most studied complement pathway

A

Classical pathway

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32
Q

The C1q is composed of how many heads?

A

6

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33
Q

How many C1q heads are needed to activate the complement?

A

At least 2

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34
Q

The stalk of the C1q is made up of which structure?

A

Collagen

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35
Q

Which ion stabilizes the C1 component?

A

Calcium

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36
Q

T/F: C2a is the lighter fragment than C2b, and will then be distributed throughout the body for biological functions

A

False (C2b is lighter than C2a, C2a will bind to C3b to produce C5 convertase)

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37
Q

In the classical pathway, what are the components of the C3 convertase?

38
Q

In the classical pathway, this cleaves the C3

A

C3 convertase/ C4b2a

39
Q

In the classical pathway, what are the components of the C5 convertase?

39
Q

Which component of the C5 convertase will bind to C5, permitting the cleavage into C5a and C5b

40
Q

What are the components of the membrane attack complex (MAC)?

41
Q

This complement pathway can also act as an “amplification loop”

A

Alternative pathway

42
Q

Other than IgM and IgG, which antibody can also activate the alternative pathway?

43
Q

The alternative pathway was first described by ___

44
Q

The stabilizing component of the alternative pathway

45
Q

In the alternative pathway, what are the components of C3 convertase?

46
Q

In the alternative pathway, what are the components of C5 convertase?

47
Q

The pathway that serves as a defense mechanism during infancy

A

Lectin pathway

48
Q

A protein that binds to carbohydrate

49
Q

What produces mannose-binding lectin?

A

Liver in acute-phase inflammatory reactions

50
Q

Identify the regulator of the complement system:

Binds C1r2s2, causing dissociation from C1q

A

C1 inhibitor

51
Q

Identify the regulator of the complement system:

Blocks the association of C4b and C2a

A

C4b-binding protein (C4bBP)
Complement receptor type I
Membrane cofactor protein (MCP)

52
Q

Inhibitor-bound C4b is cleaved by ___

53
Q

Identify the regulator of the complement system:

In alternative pathway, these will prevent the binding of C3b and factor B

A

CR1
Membrane cofactor protein (MCP)
Factor H

54
Q

Inhibitor-bound C3b is cleaved by ___

55
Q

Identify the regulator of the complement system:

Inactivates C3b and C4b, thereby regulating C3 convertase

56
Q

Identify the regulator of the complement system:

Regulates alternative pathway by targeting C3b

57
Q

Identify the regulator of the complement system:

Prevents bystander lysis

A

Decay accelerating factor/ DAF (CD55)

58
Q

Identify the regulator of the complement system:

Prevents the insertion of C5b67 MAC component into the membrane

59
Q

Identify the regulator of the complement system:

Binds C5b678, preventing assembly of poly-C9 and blocking formation of MAC

A

Homologous restriction factor (HRF)
Membrane inhibitor of reactive lysis (MIRL)

60
Q

Identify the regulator of the complement system:

Dissociates C1r and C1s from C1q

A

C1 inhibitor

61
Q

Identify the regulator of the complement system:

Cleaves C3b and C4b

62
Q

Identify the regulator of the complement system:

Cofactor with I to inactivate C3b; prevents binding of B to C3b

63
Q

Identify the regulator of the complement system:

Acts as a cofactor with I to inactivate C4b

A

C4-binding protein

64
Q

Identify the regulator of the complement system:

Prevents attachment of the C5b67 complex to cell membranes

A

S protein/ Vitronectin

65
Q

Identify the complement receptor on cell membranes:

Cofactor of factor I; mediates transport of immune complexes

A

CR1 (CD35)

66
Q

Identify the complement receptor on cell membranes:

B-cell co-receptor for antigen with CD19

A

CR2 (CD21)

67
Q

Identify the complement receptor on cell membranes:

Adhesion and increased activity of phagocytic cells

A

CR3 (CD11b/ CD18)
CR4 (CD11c/ CD18)

68
Q

Identify the complement receptor on cell membranes:

Important receptor of the Epstein-Barr virus

A

CR2 (CD21)

69
Q

Identify the regulator of the complement system:

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

A

DAF (CD55)

70
Q

Identify the regulator of the complement system:

Prevents insertion of C9 into cell membrane

A

MIRL (CD59)

71
Q

Identify the regulator of the complement system:

Cofactor for Factor I cleavage of C3b and C4b

A

MCP (CD46)

72
Q

Identify the C protein associated with the condition:

Lupus-like syndrome

A

C1r, C4 deficiency

73
Q

Identify the C protein associated with the condition:

Sex-linked agammaglobulinemia, severe combined immunodeficiency

A

C1q deficiency

74
Q

Identify the C protein associated with the condition:

Most serious, severe recurrent infections

A

C3 deficiency

75
Q

Identify the C protein associated with the condition:

Neisseria infections

A

C5-C8, MBL, Properdin deficiency

76
Q

Identify the C protein associated with the condition:

Paroxysmal nocturnal hemoglobinuria (PNH)

A

DAF, MIRL deficiency

77
Q

Identify the C protein associated with the condition:

Recurrent pyogenic infections

A

Factor H, Factor I deficiency

78
Q

Identify the C protein associated with the condition:

Pneumococcal diseases

A

MASP-2 deficiency

79
Q

C protein deficiency that has no known disease association

A

C9 deficiency

80
Q

Identify the C protein associated with the condition:

Leiner’s disease Gr (-) skin and bowel infection

A

C5 dysfunction

81
Q

Identify the C protein associated with the condition:

Erythroderma dysquamativum

A

C5 dysfunction

82
Q

Identify the C protein associated with the condition:

Reynaud’s phenomenon

A

C7 deficiency

83
Q

Identify the C protein associated with the condition:

Scleroderma like syndrome

A

C7 deficiency

84
Q

Identify the C protein associated with the condition:

Hereditary angioedema

A

C1INH deficiency

85
Q

Identify the C protein associated with the condition:

Pyogenic infections

A

Factor D deficiency

86
Q

Immunologic assay used for C1 inhibitor

87
Q

Most common assay for classical pathway that is crude screening test, measuring the amount of serum required to lyse 50% of standardized concentration of sensitized RBCs

A

Hemolytic titration (CH50) assay

88
Q

Similar to CH50 but magnesium chloride and ethylene glycol tetracetic acid are added

89
Q

In AH50, which component is removed to measure alternative pathway?

A

Calcium (since it stabilizes the C1 in classical pathway)