Term
| T or F: Most C' are made by liver hepatocytes |
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Definition
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Term
|
Definition
Classical
Alternative
Lectin |
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Term
|
Definition
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Term
|
Definition
Binds TO target (usually)
Bigger fragment |
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Term
| T or F: Complexes have enzymatic activity |
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Definition
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Term
|
Definition
1. Opsonization & phagocytosis
2. C'-Mediated cytolysis
3. Amplify immune response & inflammatory response
4. Assist in removal of immune complexes (ABY-AGN) |
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Term
| ___ plays central role in all three pathways |
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Definition
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Term
| 3 main stages of Classical pathway |
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Definition
Recognition unit
Activation unit
Membrane Attack Complex |
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Term
| Classical Recognition unit |
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Definition
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Term
| Classical Activation unit |
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Definition
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Term
|
Definition
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Term
|
Definition
C1 binds to Ig
Portion of C1 cleaves C4 into a & b fragments
C4b attaches to membrane
C2 is bound to C4b and cleaves by C1a and b fragments
This then cleaves C3 into a and b fragments |
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Term
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Definition
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Term
|
Definition
C1q binds to Fc region on Iig
C1r & C1s = enzymes which are activated by C1q binding |
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Term
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Definition
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Term
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Definition
|
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Term
|
Definition
| multiple C4bs attach for each C1 attached |
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Term
| T or F: C3 cleavage key step in complement activation for all pathways |
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Definition
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Term
| Second Amplification Step |
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Definition
| Each C3 convertase complex cleaves 200 C3 molecules |
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Term
| T or F: C3b molecules have short half-life if not bound to antigen |
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Definition
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Term
| C3b is a ____ _____ (greatly aids in phagocytosis) |
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Definition
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Term
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Definition
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Term
|
Definition
C5 convertase cleaves C5 with C5b attaching to membrane
C6-9 bind to membrane leading to cell lysis |
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Term
|
Definition
| Punch holes in cell membranes |
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Term
|
Definition
| Multiple C9 molecules form complex which is inserted into cell membrane - destroying nucleated cells / lyse any cell |
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Term
| T or F: The Classical pathway is activated by immune complexes (ABY bound to AGN) |
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Definition
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Term
| T or F: Most C' activation by microorganisms occurs through alternate pathway and lectin pathway |
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Definition
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Term
| T or F: IgG and IgM activate C' |
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Definition
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Term
| Of the two, which is more effective IgG or IgM in activating C' |
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Definition
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Term
|
Definition
Anaphylotoxin
inflamation inducing factors |
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Term
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Definition
Releases histamine
Dilates Blood Vessels
Contracts Smooth Muscles
Allows Vascular Fluid Leaks |
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Term
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Definition
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Term
|
Definition
| PMNs, monocytes, macrophages, eosinophils, B cells, T cells, follicular dendritic cells |
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Term
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Definition
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Term
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Definition
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Term
|
Definition
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Term
|
Definition
| Anaphylotoxin (weaker than C3a and C5a) |
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Term
|
Definition
| Major attractant & Strong Anaphylotoxin |
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Term
|
Definition
PMN aggregation
Lysosomal enzyme release
TNF release |
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Term
|
Definition
| Activation does NOT require ABY; Activated on microbial surfaces |
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Term
| Triggered by almost any foreign substance including bacteria, fungi, yeast, viruses, some parasites, tumor cells, bacteria containing lipopolysaccharides |
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Definition
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Term
| T or F: The alternate pathway is part of the natural defenses against infection |
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Definition
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Term
| What else is the Alternate Pathway known for |
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Definition
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Term
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Definition
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Term
| Which are not involved in the Alternate Pathway |
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Definition
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Term
|
Definition
1. Serum C3 spontaneously hydrolyzes to form tickover formed C3b
2. C2b is deposited on surface of microbe
3. Factor B binds to C3b which is attached to cell surface
4. Factor D cleaves Factor B when Factor B is bound to C3b = C3bBb
5. Ba diffused away
6. C3bBb bound by properdin
7. C3bBbC3b forms and has C5 convertase activity
8. Subsequent steps are the same as the classical (C6-C9) |
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Term
|
Definition
| Term for spontaneous hydrolysis |
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Term
| On human cells, what aborts the tickover process? |
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Definition
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Term
| T or F: Unless bound by properdin C3bBb has a short life |
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Definition
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Term
| Lectin pathway is part of the ___ ___ against infection |
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Definition
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Term
| Lectin pathway is triggered by binding of ____ ____ to ___ ___ |
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Definition
microbial polysaccharides
Circulating lectins |
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Term
|
Definition
| Proteins that bind to carbohydrates |
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Term
| Lectin pathway is activated by ___ ___ ___ found on bacterial surfaces |
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Definition
| Terminal Mannose Residues |
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Term
| 4 steps to the Lectin Pathway |
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Definition
1. Mannose-binding lectin is structurally similar to C1
2. Associated with 3 MBL serine proteases
3. MASP-2 takes active role and cleaves C4 & C2
4. Subsequent steps are the same as the classical |
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Term
| Most control proteins are aimed at halting ___ |
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Definition
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Term
|
Definition
| Blocks initiation of classical and lectin pathways |
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Term
| C1 Inhibitor is also known as |
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Definition
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Term
|
Definition
Binds C1 and dissociates portions of the complex (C1r&s from C1q)
inhibits MASP-2 binding |
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Term
|
Definition
| An immune deficiency disorder |
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Term
| Hereditary angioedema is due to |
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Definition
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Term
| Hereditary angioedema can lead to |
|
Definition
| life-threatening upper airway obstruction |
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Term
| What happens during Hereditary angioedema |
|
Definition
Excess cleavage of C4 and C2 leads to excess activation of classical pathway
Proteins produced which increase vascular permeability
Edema produced in response to localized AGN |
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Term
|
Definition
| Affects alternative and classical pathways |
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Term
|
Definition
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Term
| Factor H - alternate pathways effect |
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Definition
Prevents binding of Factor B by binding to C3b
Hits C3bBb complex |
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Term
| Factor H - both pathway effect |
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Definition
| C3b bound to Factor H is vulnerable to inactivation by Factor I |
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Term
|
Definition
|
|
Term
| What does Factor I inactivate? |
|
Definition
Inactivates C3b to iC3b when C3b bound by factor H (factor Ii and another cofactor then further breaks down)
Inactivates C4b with help from C4-binding protein (C4BP) which degrades C4b into components |
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Term
| What else does Factor I inactivate? |
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Definition
| C3b & C4b when C' components are bound to Factor H, C4BP, CR1, DAF, & another cofactor |
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Term
| T or F: C4BP + other proteins can dissociate C4b from the C4b2a complex |
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Definition
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Term
| Decay-accelerating factor present on |
|
Definition
| Peripheral blood cells, endothelial cells, and fibroblasts |
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Term
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Definition
| Dissociates components of C3 convertase: classical and alternative pathways |
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Term
| C3b and C4b bound to DAF vulnerable to inactivation by |
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Definition
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Term
|
Definition
| Protect cells from bystander lysis |
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Term
| Paroxysmal nocturnal hemoglobinuria |
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Definition
| Immune deficiency disorder |
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Term
|
Definition
| RBCs deficient in DAF & CD59 |
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Term
|
Definition
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Term
|
Definition
C3b becomes deposited on host cells
Make them a target for destruction by phagocytic cells |
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Term
|
Definition
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Term
|
Definition
| Inhibits formation of the MAC |
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Term
| CD59 binds to ___ and prevents insertion of ___ |
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Definition
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Term
|
Definition
| Interacts with C5b67 in fluid phase to prevent bystander lysis |
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Term
|
Definition
| Works with Factor I to degrade C3b, iC3b, C4b |
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Term
|
Definition
| RBC, platelets, PMNs, T cells, B cells, macrophages |
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Term
|
Definition
Opsonin receptor
Transports immune complexes to spleen and liver |
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Term
| When the immune complexes arrive what happens? |
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Definition
Immune complexes stripped off cells by tissue macrophages
RBC remain intact
Complexes proceed |
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Term
| Patients with ___, ___, ___ deficiencies at risk for lupus-like diseases |
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Definition
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|
Term
| 90% of patients without ___ or ___ develop SLE |
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Definition
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Term
| T or F: Immune complexes, if not cleared, cause tissue damage |
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Definition
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Term
|
Definition
C3b degradation products
iC3b, C3d, C3dg |
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Term
|
Definition
| B cells, follicular dendritic cells, epithelial cells |
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Term
|
Definition
| B cell stimulation; coreceptor for AGN |
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Term
|
Definition
| EBV (which causes mononucleosis) |
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Term
|
Definition
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|
Term
| Patients without CR-3 and CR-4 receptors are prone to _____ |
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Definition
|
|
Term
| 2 methods for component measurement |
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Definition
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|
Term
| C' Laboratory tests typically measure |
|
Definition
| C3, C4, and Factor B levels |
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Term
| C' Laboratory tests measure _____ NOT _____ |
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Definition
|
|
Term
| Total Hemolytic Complement (CH50) |
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Definition
Total Complement Activity
ELISA |
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Term
| CH50 provides SCREENING test for ... |
|
Definition
| the activity of the entire class complement sequence |
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Term
| ___ and ___ are acute phase reactants |
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Definition
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|
Term
| Elevated levels of ___ & ___ indicate inflammation or infections long before WBC, Sed Rate, or Fever appears |
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Definition
|
|
Term
| ___, ___, & ___ INCREASE in Acute Inflammatory Conditions (no more than 2X) |
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Definition
|
|
Term
| Elevated C' Levels in (7) |
|
Definition
SLE
Early RA
Viral Hepatitis
Acute Rheumatic Fever
Myocardial Infarction
Pregnancy
Gout |
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Term
| Decreased levels with Increased Consumption |
|
Definition
Immune complex diseases
Endotoxemia
Immune Deficiency Disorders |
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Term
| Decreased levels with Increased Consumption : ABY-AGN complexes |
|
Definition
Activate Classical pathway
May activate alternative pathway |
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Term
| Immune Complex Disease example and abnormalities |
|
Definition
Ex) Severe SLE
Ab: Decreased C3, C4, & CH50 |
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Term
| Endotoxemia (Endotoxin in blood) |
|
Definition
Lipopolysaccharide triggers alternate pathway
Factor B and C3 decreased |
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Term
| Immune Deficiency Disorders |
|
Definition
Hereditary angioedema
Paroxysmal Nocturnal Hemoglobinuria |
|
|
Term
Decreased Production
Hereditary Deficiencies |
|
Definition
Early complement component deficiencies
Late complement component deficiencies |
|
|
Term
| 2 types of Early Complement Component Deficiencies |
|
Definition
C1qr, C4, C2
C3 deficiency |
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|
Term
| Which deficiency is most serious of the Early C' Component Deficiencies |
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Definition
|
|
Term
|
Definition
| Patients have lupus-like disease |
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Term
|
Definition
Prone to developing severe recurrent infections
May be subject to immune complex diseases |
|
|
Term
| Late Complement Component Deficiencies |
|
Definition
|
|
Term
| Late Complement Component Deficiencies Recurrent |
|
Definition
| Recurrent Neisseria infections |
|
|
Term
| Decreased production other disease states |
|
Definition
Liver disease
Protein malnutrition (anorexia nervosa) |
|
|
Term
| C' can be a _____ to detect the presence of either specific ABY or AGN |
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Definition
|
|
Term
| 2 step process of C' Fixation Tests |
|
Definition
1. Test system - ABY-AGN interaction
2. Indicator system
Sheep RBC coated with ABY (hemolysin)
RBC coated with ABY lyse in presence of C' |
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Term
| C' Fixation - Procedure for detecting ABY Step 1 |
|
Definition
| Patient specimen heated at 56 degrees C for 30 minutes to inactivate any C' naturally present in patient sample |
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Term
| C' Fixation - Procedure for detecting ABY Step 2 |
|
Definition
| Patient serum serially diluted |
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|
Term
| C' Fixation - Procedure for detecting ABY Step 3 |
|
Definition
| Known AGN added to each tube + measured amount of guinea pig C' |
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Term
| C' Fixation - Procedure for detecting ABY Step 4 |
|
Definition
| If ABY present, AGN and ABYs combine and fix C' |
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|
Term
| C' Fixation - Procedure for detecting ABY Step 5 |
|
Definition
Indicator cells added (Sheep RBC coated with hemolysin)
Tubes incubated then Centrifuged |
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Term
| C' Fixation - Procedure for detecting ABY Step 6 |
|
Definition
|
|
Term
| C' Fixation Test if ABY present |
|
Definition
| ABY-AGN complexes bind C'. When indicator cells added the C' is not able to bind to indicator cells. NO HEMOLYSIS |
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Term
| C' Fixation Test if NO ABY present |
|
Definition
| ABY-AGN complexes do not bind C'. When indicator cells added the C' present and able to bind to indicator cells |
|
|
Term
|
Definition
|
|
Term
|
Definition
At some point ABY diluted out
Endpoint is highest dilution with no hemolysis |
|
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Term
|
Definition
| Performed in essentially the same way as for detecting ABYs; except USE KNOWN ABY |
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|
Term
| Quality Control is ____ _____ |
|
Definition
|
|
Term
| C' Fixation Tests are More _____ than agglutination and precipitation |
|
Definition
|
|
Term
| C' Fixation Tests are Less ____ than Labeled Assays |
|
Definition
|
|
Term
| C' Fixation Tests are ____ ___ _____ |
|
Definition
|
|
