Complement System- Exam III Flashcards

1
Q

Component of the immune system in animals that is genetically determined and nonspecific

A

Innate immune system

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2
Q

Elements of the system include mucous secretions, complement proteins, certain WBCs (especially neutrophils, macrophages, & dendritic cells)

A

Innate immune system

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3
Q

Component of the vertebrate immune system involving lymphocytes (B cells and T cells), containing a small number of genetically encoded proteins that combine to produce an enormous variety of proteins capable of recognizing and deactivating specific antigens.

A

Adaptive immune system

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4
Q

What immune system are you born with? What immune system do you acquire?

A

Innate ; adaptive

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5
Q

The first function of the complement system:

A

Alter membrane pathogens and cellular debris

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6
Q

The first function of the complement system is to alter membrane pathogens and cellular debris via:

A

Opsonization

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7
Q

What promotes removal of particles via complement receptors on host cells via a coating?

A

Opsonization

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8
Q

Opsonization also leads to the assembly of ______ on pathogen and subsequent _____.

A

MAC ; Lysis

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9
Q

MAC

A

Membrane Attack Complex

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10
Q

The second function of the complement system is to:

A

Enhance the inflammatory response

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11
Q

The complement system enhances the inflammatory response via:

A

Release of anaphpylatoxins that promote cell activation or migration to inflammatory site

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12
Q

Migration to inflammatory site

A

Chemotaxis

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13
Q

The complement system becomes activated in innate immunity when it sense:

A

Apoptotic cells, tissue debris, or pathogens

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14
Q

What level does the innate immune system function at?

A

Very low

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15
Q

The body has mechanisms in place to prevent the innate immune system from:

A

Destroying all the cells in our body

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16
Q

The complement system can be activated in 3 ways:

A
  1. C3 turnover
  2. Natural antibodies
  3. Lectins
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17
Q

Activation of the complement system by C3 turnover engages what pathway?

A

Alternative pathway

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18
Q

Activation of the complement system thought natural antibodies engages what pathway:

A

Classical pathway

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19
Q

Activation of the complement system by binding of lectins engages what pathway?

A

Lectin Pathway

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20
Q

In adaptive immunity, natural antibodies are replaced by:

A

Specific antibodies

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21
Q

Complement system triggers the following immune functions (3)

A
  1. Phagocytosis
  2. Inflammation
  3. Membrane attack
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22
Q

Phagocytosis in the complement system occurs by:

A

Opsonizing antigens

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23
Q

_____ has most important opsonizing activity

A

C3b

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24
Q

______ occurs by chemotactically attracting macrophages and neutrophils

A

Inflammation

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25
Q

What anaphylatoxins are involved in inflammation?

A

C3a & C5a

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26
Q

What process ruptures the cell wall of bacteria (punching holes in it)

A

Membrane attack

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27
Q

A local response to cellular injury that is marked by capillary dilatation, leukocytes infiltration, redness, head, and pain

A

Inflammation

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28
Q

What serves as a mechanism initiating the elimination of noxious agents and of damaged tissue?

A

Inflammation

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29
Q

Wheal and flare response is characteristic of:

A

Type 1 allergic reaction

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30
Q

Swelling produced by the release of serum into the tissues

A

Wheal

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31
Q

Redness of the skin resulting from the dilation of blood vessels

A

Flare

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32
Q

The classic pathway is triggered by:

A

The C1-complex

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33
Q

The mannose binding (lectin) pathway is homologous to the classical pathways but uses the opsonins ______ and ______ instead of C1q

A

Mannose-binding lectin (MBL) & Ficolin

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34
Q

What opsonin is used in the classical pathway

A

C1q

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35
Q

The alternative pathway is continuous activated at a _____ level.

A

Low level

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36
Q

The alternative pathway is continuously activated at a low level as a result of:

A

Spontaneous C3 hydrolysis

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37
Q

In the alternative pathway spontaneous C3 hydrolysis occurs as a result of:

A

Breakdown of the internal thioester bond

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38
Q

The breakdown of the internal thioester bond is due to C3 being mildy unstable in:

A

Aqueous environment

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39
Q

Pathway that does not rely on pathogen-binding antibodies like other pathways

A

Alternative pathways

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40
Q

Which pathway responds when you have antigen:antibody complexes (on pathogen surfaces)

A

Classical pathway

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41
Q

The classical pathway involves what type of molecules? (3)

A

C1, C4, C2

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42
Q

The C1 molecule in the classical pathway breaks down into:

A

C1q, C1r, C1s

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43
Q

Pathway involving mannose binding lectin or ficolin binding carbohydrates on pathogen surfaces

A

Lectin Pathway

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44
Q

Lectin pathway involves mannose-binding lectin or ficolin binding to _____ on pathogen surfaces

A

Carbohydrates

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45
Q

What molecules does the election pathway involve?

A

MBL/ficolin, C4 and C2

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46
Q

The enzymatic activity of the MBL/ficolin complex in the lectin pathway:

A

MASP-2

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47
Q

What molecules do the lectin and classical pathway have in common?

A

C2 & C4

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48
Q

The alternative pathway occurs on:

A

Pathogen surfaces

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49
Q

The molecules involved in the alternative pathway include:

A

C3, B and D

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50
Q

All 3 pathways converge at the production of:

A

C3 convertase

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51
Q

What two pathways are pretty much identical except for what triggers them?

A

Classical and lectin pathway

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52
Q

A molecule that cleaves different members of the complement pathways

A

C3 convertase

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53
Q

C3 convertase cleaves C3 into:

A

C3a and C3b

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54
Q

After C3 convertase cleaves C3 into C3a and C3b, the C3b will then bind:

A

C3 convertase

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55
Q

When the C3 convertase and C3b bind together, this leads to the cleavage of:

A

C5 into C5a and C5b

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56
Q

What molecules are peptide mediators inflammation and phagocyte recruitment?

A

C3a & C5b

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57
Q

What molecule binds to the complement receptors on phagocytes and leads to the opsonization of pathogens and removal of immune complexes

A

C3b

58
Q

What molecule triggers MAC being formed along with lysis of certain pathogens and cells

A

C5b

59
Q
  1. Molecule important in opsonization:
  2. Molecule important in MAC formation:
  3. Molecules important in inflammation:
A
  1. C3b
  2. C5b
  3. C3a & C5a
60
Q

In addition to the C3 being broken down into C3a and C3b during activation of complement system, our bodies also

A

Constituitively hydrolyze C3 into C3a and C3b but at a very low level

61
Q

The central component of the complement system:

A

Complement fixation

62
Q

In fixation of complement what molecule tags the bacterium for destruction?

A

C3b

63
Q

In the fixation of the complement system, what molecule recruits phagocytes & functions in inflammation

A

C3a

64
Q

The cleavage of C3 exposes:

A

Thioester bond

65
Q

When the thioester bond is exposed on C3, this allows ____ to strongly covalently bind

A

Nucleophiles

66
Q

In the classical pathway of complement, you have antibodies coating the antigen surface and the _____ molecule is going to bind to that

A

C1 molecule made of C1q+r+s

67
Q

What component of the C1 molecule will cleave C4 into C4a and C4b?

A

C1s

68
Q

The cleavage of C4 into C4a and C4b will induce the cleavage of _____ into _____.

A

C2 into C2a and C2b

69
Q

C4b and C2a together will cleave C3 into:

A

C3a and C3b

70
Q

C4b+C2a =

A

C3 convertase

71
Q

C1 is a complex of C1q, C1r, and C1s. The C1q portion is composed of:

A

6 identical subunits with globular heads and long collagen-like tails

72
Q

The head-like structures of C1q function to:

A

Sit down and bind pathogen surface

73
Q

Once the C1q globular heads are bound to the pathogen surface, the C1r portion undergoes a conformational change which:

A

Cleaves and activates C1s zymogen

74
Q

C1s will bind to the

A

Receptors on the pathogen surface

75
Q

What cleaves C4 into C4a and C4b?

A

Activated C1s

76
Q

When the C4b binds to C2, the C2 will be cleaved by ____ to form C2a and C2b

A

C1s

77
Q

After the C2 is cleaved into C2a and C2b, _____ combines with theC4b to create ____.

A

C2b to create C3 complement (C4bC2a)

78
Q

Because the C4bC2a is an active C3 convertase, it functions to cleave:

A

C3 into C3a and C3b

79
Q

After C3 is cleaved by the C3 convertase into C3a and C3b, it will bind either:

A

Microbial surface or the convertase itslef

80
Q

One molecule of C4b2a can cleave up to _____ molecules of C3 into C3b.

A

1000

81
Q

The whole process starting with the C1s binidng to the microbial surface and ending with the cleavage of C3 and all the in between steps are part of the :

A

Opsonization process

82
Q

When molecules get coated with _____ they are said to be opsonized.

A

C3b

83
Q

In the mannose lectin binding pathway, instead of the antibody binding event, it uses:

A

Lectin binding proteins

84
Q

Mannan-binding lectin forms a complex with ______ that resembles the complement C1 complex.

A

Serine proteases

85
Q

MLB forms cluster of two to six _______ heads around a central _____ like stalk.

A

Carbohydrate binding heads; collagen-like

86
Q

What are the 2 associated enzyme activities of the two serine proteases in mannan-binding lectin?

A

MASP1 and MASP2

87
Q

The function of the MASP1 & 2 enzymes associated with Mannan-binding lectin allows for the cleavage of:

A

C4 and C2

88
Q

Mannan-binding lectin and ficolin both are associated with:

A

MASP-1 and MASP-2

89
Q

in the mannose-binding lectin pathway, the C4b2a is a C3 convertase; it will cleave the C3 into C3a and C3b and the C3b will bind the the C4b2a complex generating:

A

C5 convertase

90
Q

In the alternative pathway: The C3b is deposited by the classical pathway or lectin pathway’s:

A

C3 convertase

91
Q

In the alternative pathway: The C3b binds to _____.

A

Factor B

92
Q

In the alternative pathway: The bound factor B is cleaved by _____ into Ba and Bb

A

Plasma protease factor D

93
Q

The C3bBb complex is a:

A

C3 convertase

94
Q

The alternative pathway of complement involves creating a C5 convertase that ultimately leads to the deposition of:

A

MAC complexes

95
Q

In addition to the alternative pathway, the C5 convertase can also be generated by other pathways, the key is that you need to generate a _____ in order to create a convertase that will cleave C5 into C5a and C5b

A

C3b complex

96
Q

The alternative pathway is triggered by the:

A

Covalent binding of C3b to pathogen or cell surface

97
Q

In the alternative pathway, what forms the pore on the membrane surface?

A

C9

98
Q

The MAC complex is composed of molecules:

A

C5b, C6, C7, C8, and a bunch of C9

99
Q

All three pathways lead up to:

A

MAC complex

100
Q

What is the most direct path leading to the MAC complex?

A

Alternative pathway

101
Q

During the process of opsonization and phagocytosis, the phagocytic cell expresses a specific receptor for the ___________ that are on the surface

A

Complement molecules

102
Q

If the Phagocytic cells don’t have the complement receptor receptor specific to the complement molecules coating the pathogen they:

A

Will not be able to phagocytose the molecule

103
Q

You can opsonize and coat the bacteria with C3b, bind them to the complement receptor on the phagocytic cell, but if ____ is not present, the bacteria will not be internalized by the phagocytic cell

A

C5a

104
Q

All of the cells in our body contain different proteins on their surfaces in aims to prevent the complement pathway:

A

From attacking self-cells

105
Q

Membrane protein that displaces Bb from C3b and C3b from C4b:

A

Decay-accelerating factor (DAF)

106
Q

Membrane proteins that promotes C3b and C4b in activation by I

A

Membrane cofactor protein (MCP)

107
Q

DAF and MCP are both:

A

Control proteins

108
Q

We have spontaneous production of ____ at low levels.

A

C3b

109
Q

If we C3b binding to autologous host membranes it will interact with _____ which blocks the association of factor B with C3b thereby preventing the formation of _____.

A

DAF; C3 convertase

110
Q

On the cell membrane, DAF causes the C3 convertase to ______ .

A

Dissociate

111
Q

MCP binding to the C3bBb complex causes the release of the _____ portion, and following, a ______ molecule will come in and bind to the C3b cleaving it into an inactive form.

A

Bb portion; factor I molecule

112
Q

MCP and DAF both involve the dissociation of the Bb portion, but MCP additionally makes the C3b susceptible to:

A

Cleavage by facto I

113
Q

Factor I works to:

A

Cleave C3b

114
Q

Diseases that are involved in the complement pathway boil down to: (2)

A
  1. Inappropriate activation of the complement pathway
  2. Destruction of self cells
115
Q

Acquired disorder that results in premature death and impaired production of blood cells.

A

Paroxysmal nocturnal hemogloburinuria

116
Q

What cells does PNH affect?

A

RBC, Leukocytes, and platelets

117
Q

Who does PNH effect and when does diagnosis typically occur?

A

Both sexes equally, and young adulthood

118
Q

Paroxysmal nocturnal hemoglobinurina results from:

A

Deficiency in the DAF protein

119
Q

PNH is named based on:

A

It occurring at night

120
Q

In PNH, the deficiency of DAF protein:

A

Results in destruction of self cells

121
Q

Results from chronic uncontrolled activation of the complement system

A

Atypical hemolytic uremic syndrome

122
Q

Disease in which multiple blood clots form throughout the body in small blood vessels which can lead to stroke, heart attack, kidney failure, and death

A

Atypical hemolytic uremic syndrome

123
Q

Atypical hemolytic uremic syndrome is due to:

A

Mutations in factor H, Factor I, or membrane cofactor protein

124
Q

The most common functional defect in atypical hemolytic uremic syndrome in age-related macular degeneration syndrome:

A

Reduced cofactor activity (MCP or Factor H) for C3b

125
Q

Atypical hemolytic uremic syndrome is a very ______ disease in terms of which proteins are affected, which also determines the _____ of the disease.

A

Heterogenous; severity

126
Q

In atypical hemolytic uremic syndrome, the most commonly affected factor causing the disease is:

A

Factor H

127
Q

In normal conditions you have MCP or factor H that will bind to the C3b when C3b is created, and this is where factor I comes in and cleaves C3b into an inactive form. If you don’t have this factor H or MCP molecule, then you wont be able to bind _____.

A

Factor I

128
Q

If there is spontaneous creation of the C3b from the C3 convertase or from hydrolysis that occurs naturally, these molecules that are our defense mechanism are there to:

A

Prevent initiation of the complement cascade

129
Q

Disease characterized by recurrent attacks of severe swelling

A

Hereditary angioedema

130
Q

Angioedema caused by mutations in the gene that makes the C1 inhibitor

A

Type 1 and Type 2 angioedema

131
Q

Hereditary angioedema where there is a deficiency of C1

A

Type 1

132
Q

Hereditary angioedema characterized by an atypical C1 proteins that is less capable of suppressing activation of the complement system

A

Type 2 HA

133
Q

Hereditary angioedema characterized by mutations in the Factor XII gene

A

Type 3 HA

134
Q

Factor XII is present in the:

A

Clotting cascade

135
Q

Factor XII gets cleaved into Factor XIIa also known as:

A

Plasmin

136
Q

Factor XIIa (Plasmin) functions to cleave prekalikrein into:

A

Kalikrein

137
Q

Factor XIIa also function in the conversion of the _____ pathway in the clotting cascade

A

Common

138
Q

Kalikrein converts a high-molecular-weight Kinninogen into Bradykinin and the bradykinin plays a role in: (3)

A
  1. Vasodilation
  2. Smooth muscle contraction
  3. Edema
139
Q

The classic pathway works through what type of complexes?

A

Antigen-antibody complexes

140
Q

The lectin pathway and alternative pathway function within the _______ immune system, whereas the classical pathway functions with ______ immune system.

A

Innate immune system

Both innate and adaptive