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SGU Immuno # 5 - Complement
SGU Immuno # 5 - Complement
55
Medical
Professional
12/23/2012

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Term
Complement proteins are synthesized where?
Definition
In the liver
Term
What is common b/w the alternative and classical complement pathways?
Definition
Both pathways generate the "C3b" fragment
Term
What complement pathway leads to osmotic lysis of cells or microbes?
Definition
Terminal pathway of complement cascade ; this is done by MAC complex
Term
Complement component C3b can bind to what in circulation ?
Definition
Immune complexes
Term
How many base complement proteins are there?
Definition
C1 to C9
Term
When is classical complement pathway initiated ?
Definition
Only when IgM or IgG Abs bind to antigen or when antigen/antibody complex (immune complex is formed)
Term
In an immune complex what is bound to Ab and what is bound to complement ?
Definition
Antigen is bound via Fab region of Ab
Fc region binds to complement protein
Term
C1 is composed of what 3 components?
Definition
C1q, C1r, and C1s
Term
Why is IgM more efficient at complement activation than IgG ?
Definition
IgM is a pentamer
Term
What is the classical complement pathway C3 convertase ?
Definition
The C4b2a complex
Term
What does the C3 convertas complex in classical complement pathway do ?
Definition
It cleaves C3 to C3a and C3b
Term
What is hallmark of classical complement pathway activation ?
Definition
Formation of C3 convertase
Term
What is required for C5 convertase formation in classical complement pathway ?
Definition
C3b
Term
What is the C5 convertase and what does it do?
Definition
C4b2a3b ; it cleaves C5 into C5a and C5b
Term
What are C3a and C5a ?
Definition
Are anaphylatoxins
Bind to mast cells and basophils, induce degranulation, and release of histamine and other inflammatory mediators
Term
Function of histamine is ?
Definition
Increases vascular permeability
Term
C5a is chemotactic for what?
Definition
Phagocytes
Term
When is anaphyhlatoxin C4a generated ?
Definition
Only following activation of the classical complement pathway
Term
What are the many function of C3b ?
Definition
It serves as an opsonin on microbial surfaces
Facilitates elimination of immune complexes
Amplifies complement activity
is required for formation of C5 convertases in both alternative and complement pathways
Term
MAC is formed by what complement proteins ?
Definition
C5b, C6, C7, C8, C9
Term
What does C2b do ?
Definition
Is a weak kinin that increases vascular permeability , and so contributes to genesis of inflammation
Term
What is the C3 convertase in alternative pathway ?
Definition
C3bBbC3b
Term
What is the C5 convertase in terminal pathway ?
Definition
C5b, 6, 7, 8, 9
Term
Components of the Terminal / Common Complement pathway ?
Definition
C5b, C6, C7, C8, C9
Term
What is the initiator molecule for generation of MAC in the terminal complement pathway ?
Definition
C5b
Term
What are the complement proteins in the alternative complement pathway ?
Definition
C3b, Factor B, Factor D, Properdin, C3, C5
Term
How is alternative pathway activated ?
Definition
Invasion of an infectious agent causes spontaneous creation of C3b (or "tickover")
Term
What happens to spontaneously generated C3b in absence of microbial infection ?
Definition
C3b is rapidly inactivated either by interaction with water or complement regulatory proteins
Term
What does Factor D do ?
Definition
Causes hydrolysis of Factor B to Ba and Bb
Term
What is the alternative pathway C3 convertase ?
Definition
C3bBb
Term
What stabilizes the C3 convertase in the alternative complement pathway ?
Definition
Properdin
Term
What is the alternative pathway C5 convertase ?
Definition
C3bBbC3b
Term
C1 INH (C1 esterase inhibitor) does what ?
Definition
Inhibits C1 and the protease kallikrein
Term
Kallikrein links what two things ?
Definition
The complement and intrinsic coagulation pathways
Term
What does DAF (Decay Accelerating Factor) do ?
Definition
Blocks formation of alternative and classical pathway C3 convertases, by binding to C4b and C3b
Term
What does C4bp (C4 binding protein) do ?
Definition
Is a cofactor for Factor I
Binds to C4b preventing its attachment to cells
Term
What does Factor I do ?
Definition
Factor I cleaves C4b to its inactive form C4bi
Term
What does AI (Anaphylatosin inhibitor) do ?
Definition
Binds to C3a, C4a, and C5a and inhibits their binding to receptors on mast cells and basophils
Term
What are the classical complement pathway regulators ?
Definition
C ACFD
C1 INH
AI
C4bp
Factor I
DAF
Term
What are the alternative complement pathway regulatory proteins ?
Definition
FFAD
Factor H
Factor I
AI
DAF
Term
What does Factor I do ?
Definition
Inactivates C3b/C4b via CR1
Term
What does Factor H do in alternative pathway?
Definition
Competitively binds with C3b, inducing dissociation of this convertase
Term
What are the 3 MAC inhibitors ?
Definition
HRF (homologous restriction factor)
S-protein / vitronectin
CD59 / HRF20
Term
What does kallikrein do ?
Definition
In presence of cofactor kininogen, Factor 12 is converted to Factor 12a
Term
Do Factor 12a and kallikrein reciprocally activated each other ?
Definition
Yes
Term
Proteolytic hydrolysis of kininogen does what ?
Definition
Generates kininogen(a) and bradykinin (a vasodilator that increases vascular permeability).
Term
What is the complement ligand fragment for CR1 receptor ?
Definition
C3b and C4b
Term
CR1 is found on what cells ?
Definition
Phagocytes and Erythrocytes
Term
CR1 binds with C3b or C4b to do what ?
Definition
Opsonin mediated recognition of antigen
Clearance of circulating immune complexes
Term
CR2 receptor binds with what complement ligand fragment ?
Definition
C3bi
Term
CR2 receptor is found on what cell type ?
Definition
B lymphocytes
Term
CR2 binding with C3bi occurs in what mode of infection ?
Definition
Mode of Epstein Barr virus infection
Term
CH50 complement test is for what ?
Definition
Classical complement pathway
Term
If CH50 test is not available, then what is done ?
Definition
Tests for C3 and C4
Term
Does CH50 test detect deficiencies in specific complement components ?
Definition
No, it measures total classic complement pathway activity
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