Complement Flashcards

1
Q
A
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2
Q
A
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3
Q

MBL function

A

Binds to mannose

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4
Q

MBL pathway

A

Lectin

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5
Q

MBL associated diseases

A

Mostly asymptomatic
Bacterial infections
Some autoimmunity

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6
Q

MASP-1 Function

A

Unknown

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7
Q

MASP-1 pathway

A

Lectin

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8
Q

MASP-1 associated diseases

A

3MC syndrome

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9
Q

MASP-2 function

A

Cleaves C4 and C2

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10
Q

MASP-2 pathway

A

Lectin

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11
Q

MASP-2 associated diseases

A

Mostly asymptomatic
Respiratory infections

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12
Q

Factor B function

A

Binds to C3b to form C3 convertase

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13
Q

Factor B pathway

A

Alternative

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14
Q

Factor B associated diseases

A

Neisseria and pneumococcal infections
aHUS with gain of factor

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15
Q

Factor D function

A

Cleaves factor B

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16
Q

Factor D pathway

A

Alternative

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17
Q

Factor D associated diseases

A

Bacterial infection

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18
Q

Properdin function

A

Stabilizes C3bBbC3 convertase

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19
Q

Properdin pathways

A

Alternative

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20
Q

Properdin associated diseases

A

Severe recurrent infections

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21
Q

C3 function

A

Key intermediate in all pathways

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22
Q

C3 pathways

A

Terminal

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23
Q

C3 associated diseases

A

Severe recurrent infections
aHUS, C3G, and AMD with gain of function

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24
Q

C5 function

A

Initiates MAC

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25
Q

C6 function

A

Binds to C5b in MAC

26
Q

C7 function

A

Binds to C5bC6 in MAC

27
Q

C8 function

A

Starts pore formation

28
Q

C5, C6, C7, C8 pathway

A

Terminal

29
Q

C5, C6, C7, C8 associated disease

A

Neisseria infection

30
Q

C9 function

A

Polymerizes to cause cell lysis

31
Q

C9 pathways

A

Terminal

32
Q

C1q Function

A

Binds to Fc region of IgM and IgG
Synaptic pruning

33
Q

C1r function

A

Activates C1s

34
Q

C1s function

A

Cleaves C4 and C2

35
Q

C1 pathway

A

Classical

36
Q

C1 associated diseases

A

SLE
Recurrent infections

37
Q

C4 function

A

Part of C3 convertase

38
Q

C4 pathway

A

Classical

39
Q

C4 associated diseases

A

Often asymptomatic
Rheumatoid arthritis
SLE
Infection

40
Q

C2 function

A

Binds to C4b to form C3 convertase

41
Q

C2 pathway

A

Classical

42
Q

C2 associated diseases

A

SLE
recurrent infections
May be asymptomatic

43
Q

The classical pathways is activated primarily by

A

Antigen-antibody complexes

44
Q

Name a characteristic of complement components

A

Normally present in serum

45
Q

Recognition unit

A

Consists of C1qrs
The subunits require calcium for binding
Binding occurs at the Fc region of antibody molecules

46
Q

Synonyms for C3 convertase

A

C4bC2a
C3bBb

47
Q

Mannose binding protein in the lectin pathway is most similar to what classical pathway component?

A

C1q

48
Q

Role of Properdin in the alternative pathway

A

Stabilization of C3 convertase

49
Q

MAC

A

C9 polymerizes to form the transmembrane channel

50
Q

Functions of complement system

A

Lysis of foreign cells
Increase in vascular permeability
Migration of neutrophils to the tissues

51
Q

Diseases associated with complement deficiencies or improper control

A

AMD
PNH
aHUS
C3 glomerulopathy

52
Q

Amplification loop

A

Improper control can lead to disease
It can amplify activation that is initiated by the classical pathway
C3b is the product that is increased

53
Q

Factor H acts by competing with ___ for the same binding site

A

Factor B

54
Q

If a specimen for complement testing was left on a laboratory bench overnight, and not properly frozen, what would happen?

A

A CH50 level would be falsely low

55
Q

A lack of C1-INH has been associated with

A

Hereditary angioedema

56
Q

Cofactors for Factor I

A

Membrane cofactor protein
C4-binding protein
Factor H

57
Q

Anaphylatoxin

A

Increases permeability of blood vessels

58
Q

A decreased in CH50 level and a normal AH50 level indicates

A

Decrease in components of the classical pathway

59
Q

The CH50 test measures

A

Patient serum required to lyse 50% of sensitized sheep RBCs

60
Q

HUS

A

Common cause of renal failure in children

61
Q

What would be expected of a patient on a complement C5 inhibitor

A

Low CH50 and AH50

62
Q

The role of CR2 on cell membranes

A

Acts as co-receptor on B cells for antigen