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Understanding the Complement System

The document outlines the complement system's role in immune response, detailing its activation pathways, key components, and outcomes such as vasodilation, chemotaxis, opsonization, and the formation of the membrane attack complex (MAC). It emphasizes the importance of proteins like C3 and C5 in these processes and discusses how pathogens can evade the immune response. Additionally, it touches on T cell activation and the role of major histocompatibility complex (MHC) proteins in presenting antigens to T cells.

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Meseret Workie
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0% found this document useful (0 votes)
7 views68 pages

Understanding the Complement System

The document outlines the complement system's role in immune response, detailing its activation pathways, key components, and outcomes such as vasodilation, chemotaxis, opsonization, and the formation of the membrane attack complex (MAC). It emphasizes the importance of proteins like C3 and C5 in these processes and discusses how pathogens can evade the immune response. Additionally, it touches on T cell activation and the role of major histocompatibility complex (MHC) proteins in presenting antigens to T cells.

Uploaded by

Meseret Workie
Copyright
© All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd

Complement Activity

Lesson objectives
At the end of this lesson student will be able to:
 List down the important steps in the complement
system
 Recognize the importance of chemotaxis in the
complement system
 Appreciate the role of the membrane attack
system
in immune response

09/15/2025 Advanced Immunology 1


Complement Activity

 Complement activation is a cascade of events

 The Complement cascade consists of a series of


protein proenzymes, present in the blood, that
are converted to active enzymes by interaction
with pathogens and with each other

 The Complement cascade has a number of very


important roles in combating infection and in
inflammatory disease.

09/15/2025 Advanced Immunology 2


Complement Activity...

 There are two major ways of activating the


Complement cascade (are three)

 by the “classical pathway” or the “alternate” pathway.

 The initial components of the classical pathway


(C1,C4,C2) are activated by the Fc regions of IgG or IgM
antibody, but only after antibody has bound to antigen
(usually to a pathogen).

 The initial components of the alternate pathway (Factor


B, D and P) are directly activated by certain pathogens

09/15/2025 Advanced Immunology 3


Complement Activity...

 Either pathway can be partly activated by blood coagulation.


 There are four major outcomes of activating this cascade:
1. local vasodilation and increase in vascular permeability

2. attraction of immune cells, especially phagocytes


(chemotaxis)

3. opsonization (or tagging) of foreign organisms for


phagocytosis

4. destruction of invading organisms by the membrane attack


complex (MAC attack)
09/15/2025 Advanced Immunology 4
Complement Activity...

 Although all the molecules in the cascade are


important, the third component, called C3 protein,
stands out as of special interest because both the
classical and alternate pathways converge on C3.

 It is an enzyme that is split into two fragments (C3a


and C3b) by components of either the classical
pathway (C4b, C2a) or the alternative pathway
(factor Bb, C3b, and P).

09/15/2025 Advanced Immunology 5


Complement Activity...

 C3a is primarily a very powerful activator of mast cells


to release mediators which cause local vascular
permeability and vasodilation.

 C3a is also a chemotactic factor for phagocytic


immune cells.

 C3b, on the other hand, binds directly to the surface of


foreign organisms (or immune complexes) and acts to
'tag' these for destruction by phagocytic cells like
macrophages and neutrophils which have specific
receptors for C3b.
09/15/2025 Advanced Immunology 6
Complement Activity...

• C3b also reacts directly with the other


components
of the Complement cascade (C5-9) to produce
the MAC attack.

09/15/2025 Advanced Immunology 7


Complement Activity...

• Complement is triggered by antibodies or invading


organisms, and that the end result of the pathway
becoming activated is:
1. vasodilation and increased vascular permeability

2. attraction of immune cells to the site

3. opsonization for phagocytosis

4. interacting with other components of Complement


to initiate the formation of the MAC attack
09/15/2025 Advanced Immunology 8
Complement Activity...

09/15/2025 Advanced Immunology 9


C1qr2s2 COMPLEX

09/15/2025 Advanced Immunology 10


Complement-Induced Vasodilation and
Increased Vascular Permeability
 The Complement components responsible for
vasodilation, and vascular permeability, are the "a"
subunits of C3, C4 and C5 (C3a, C4a and C5a).

 These three proteins are called anaphylatoxins.


There are receptors for these proteins on the
surface of mast cells and basophils.

 Binding of the anaphylatoxins to their receptors


induces the cell to degranulate, releasing
mediators like histamine.

09/15/2025 Advanced Immunology 13


Complement-Induced Vasodilation and
Increased Vascular Permeability...

• Anaphylatoxin is a substance composed of the C3


and C5 components of the complements which
cause vasoactive mediators to be released from
mast cells, promoting vascular permability and
the formation of edema during an anaphylatic
reaction

09/15/2025 Advanced Immunology 14


Complement-Induced Vasodilation and
Increased Vascular Permeability...

 Such mediators induce smooth-muscle dilation on


the arteriolar side in the vasculature making the
blood vessels wider and allowing more blood to
flow to the capillaries in the area.

 They also result in an increase in the permeability


of the endothelial lining of the blood vessels.

 Increased vascular permeability allows fluid as


well as macromolecules like IgG and Complement
to flow out of the blood plasma into the tissue.
09/15/2025 Advanced Immunology 15
Complement-Induced Vasodilation and
Increased Vascular Permeability...

 Vasodilation results in redness and increased


permeability results in swelling (edema).

 The advantage of these functions of the


Complement system is to increase blood flow to the
site of infection and allow antibodies, more
Complement and immune cells to enter the tissues
to "scale up" the attack.

09/15/2025 Advanced Immunology 16


Chemotaxis

 Chemotaxis is the process whereby an immune cell


is attracted to, and moves toward, a soluble factor.

 Usually the cell being attracted is a phagocyte (like


a neutrophil).

 One of the most potent chemotactic agents is C5a.

 C5a is formed when the Complement component


C5 is cleaved to form C5a and C5b.

09/15/2025 Advanced Immunology 17


Chemotaxis

 Other substances that have chemotactic ability


are secreted by immune cells and released during
inflammatory responses.

 A number of these are small protein molecules


called “chemokines”.

09/15/2025 Advanced Immunology 18


Chemotaxis...

 Chemotaxis of phagocytic cells is important


because it "recruits" the cells to the tissue where
they are needed to ingest the invading organism
or antigens or debris from the inflammation.

 It is important to remember that immune cells do


not “leak out” of the blood because of increased
vascular permeability like plasma proteins and
water do.

09/15/2025 Advanced Immunology 19


Chemotaxis...

 They must be attracted to the site by chemotaxis


and penetrate through the endothelial lining of
the vasculature in an active process of
“transmigration”.

 After they transmigrate they essentially “crawl”


through the extravascular tissue to the site of the
infection or inflammation

09/15/2025 Advanced Immunology 20


Opsonization

 Opsonization is the process where particles such


as microorganisms become coated with
molecules which bind to specific receptors on
phagocytes.

 IgG and Complement proteins like C3b can


opsonize and are therefore referred to as
"opsonins“.

09/15/2025 Advanced Immunology 21


Opsonization ...

 The Complement fragment C3b non specifically


binds to foreign organisms or immune complexes
during Complement activation.

 Since phagocytes have receptors for C3b on their


surface (CR3) the binding of C3b to a microorganism
or an immune complex tags it for ingestion and
degradation by the phagocytes

09/15/2025 Advanced Immunology 22


Membrane Attack Complex -MAC

 Complement activation by either the classical or


alternative pathway will result in the formation of
the membrane attack complex or MAC.

 The last steps in the Complement cascade, after


the activation of C3b, involve C5b, C6, C7 C8 and
C9 which complex to form the MAC.

09/15/2025 Advanced Immunology 23


Membrane Attack Complex -MAC

 Briefly, C5 is broken down into C5a and C5b after


binding to C5 convertase, an enzyme formed by
other fragmented Complement proteins.

 C5a diffuses away and has anaphylatoxin and


chemotactic activity

09/15/2025 Advanced Immunology 24


Membrane Attack Complex –MAC..

 The C5b fragment binds directly to the target


organism and becomes the binding site for other
Complement components.

 C5b will quickly degrade unless C6 binds to it


creating C5b6, to which C7 will then bind.

09/15/2025 Advanced Immunology 25


Membrane Attack Complex –MAC..

 The C5b fragment binds directly to the target


organism and becomes the binding site for other
Complement components.

 C5b will quickly degrade unless C6 binds to it


creating C5b6, to which C7 will then bind.

 The addition of C7 changes the conformation of


the proteins so that they are able to insert into
lipid membranes.

09/15/2025 Advanced Immunology 26


Membrane Attack Complex –MAC..

 Next, C8 and finally C9 bind to the complex


creating the MAC.

 C5b678 will bind up to 16 molecules of C9.

 The MAC looks like a tube with a pore in the


centre (like a very short straw)

09/15/2025 Advanced Immunology 27


Membrane Attack Complex –MAC..

 Because of its structure and hydrophobic nature,


the MAC inserts into the membrane of the
organism or cell and allows ions, water and other
small molecules to freely pass through the pore
(the MAC attack).

 As a result, the organism will not be able to


maintain osmotic integrity and will quickly die.

 Cells without a cell wall will often burst (lyse).

09/15/2025 Advanced Immunology 28


They Fight Back

 On a closing note it is important to recognize that


the immune response to pathogens is like a war
and we don’t always win easily.

 The pathogens have ways of avoiding our


defenses. Some microbes evade destruction by
having surfaces that interfere with opsonization by
C3b or insertion of the MAC.

 Others inhibit Complement activation (they have


proteins that bind IgGFc such as protein A and
protein G).
09/15/2025 Advanced Immunology 29
They Fight Back

 Still others cause Complement degradation by


elaborating enzymes that degrade Complement
components.

 Not surprisingly, such microbes are pathogenic


(meaning they cause disease).

09/15/2025 Advanced Immunology 30


T Cells

 It is very difficult to discuss T cell activation


without knowledge of the presentation of antigen
by protein molecules coded for by the genes in
the
major histocompatibility complex.

 These molecules, MHC Class I and MHC Class II


are pivotal for T cell activation.

09/15/2025 Advanced Immunology 31


T cells..

 MHC stands for major histocompatibility complex.

 In humans the same proteins are often referred to


as human leukocyte antigens or HLA.

 Since MHC is a more specific term (there are lots


of antigens on human leukocytes, not all of them
are MHC antigens) it will be used in this resource.

09/15/2025 Advanced Immunology 32


MHC-Class I

 All cells in the body except red blood cells have


MHC class I protein on the surface.

 Your finger cells, your liver cells, your B cells, your


gut cells - they all have MHC class I on the
surface. Everyone has MHC class I proteins but
only identical twins will have identical MHC class
I.

09/15/2025 Advanced Immunology 33


MHC-Class I

 The genes for the MHC proteins show considerable


diversity between people.

 Class I MHC is a complex of the Class I protein and


another protein called β2 microglobulin

 Since everyone has a different MHC, this allows your


body to tell what cells belong to it and what cells are
foreign.

 The function of MHC-I is to sample the internal contents


of the cell and show them to the immune system.

09/15/2025 Advanced Immunology 34


MHC-Class II

 Class II MHC is a complex of proteins only


expressed by antigen presenting cells (APC).

 The function of these proteins is to present an


antigen to T helper cells to activate an immune
response which will provide both humoral
(antibody) and cell mediated immunity.

 The class II MHC consists of an alpha and a beta


chain with a transmembrane segment to hold them
on the surface of the cell.
09/15/2025 Advanced Immunology 35
MHC-Class II

 At the end farthest from the cell is a cleft where


the processed antigen sits.

 The processed antigen consists of a small peptide


of about 13-16 amino acids.

 MHC-II picks up the antigen that has been


ingested (phagocytosed) by the APC.

09/15/2025 Advanced Immunology 36


MHC structure

Peptide binding cleft Peptide binding cleft

2 1 1 1
3 2-microglobin 2 2

Class I MHC Class II MHC

 MHC classes I and II have an almost identical 3-D structure.

 Both classes of MHC are polygenic (each cell has many MHC genes) and
polymorphic (there are many alleles for each locus), but the MHC genes do
not undergo recombination.

Note: Human MHC are called HLA (human leukocyte antigen).


09/15/2025 Advanced Immunology 37
T cells

 T cells are lymphocytes which develop in the


thymus.

 T cells have antigen recognition molecules (T cell


receptor, TcR) on the surface which are composed
of two different polypeptide chains; alpha and
beta chains.

09/15/2025 Advanced Immunology 38


T cells

 The alpha and beta chains are associated with a


group of five linked proteins collectively referred
to as CD3.

 A minor population of T cells has two different


chains, a gamma chain and a delta chain

09/15/2025 Advanced Immunology 39


T cells...

 Little is known of the activities of these gamma-


delta T cells.

 Like antibody, the TcR has both a variable and a


constant region.

 As described for antibodies, the variable region of


the TcR is created by gene rearrangement.

09/15/2025 Advanced Immunology 40


T cells...

 The “T cell receptor complex” includes the alpha


and beta chain of the TcR and the associated CD3
proteins.

 The recognition of antigen-MHC by the TcR-CD3


complex does not require any other molecules.

09/15/2025 Advanced Immunology 41


T cells...

 However, other proteins on T cells are important


in the interaction leading to T cell activation.

 These "accessory " or “co-stimulatory” molecules


on T cells bind to ligands (surface molecules
which function as a lock and key binding pair) on
antigen presenting cells to give the 'second
signal' required for T cell activation.

09/15/2025 Advanced Immunology 42


T cells...

 The TcR will only recognize antigen as a peptide


in the groove of MHC.

 This is because part of the TcR actually binds to


part of the MHC molecule during recognition.

 The TcR will not bind to free antigen.

09/15/2025 Advanced Immunology 43


T cells...

 Recent evidence also suggests that some T cells can


recognize antigen presented by a molecule called CD1.

 This molecule is similar in general shape to Class I MHC


but is capable of presenting lipid and carbohydrate
antigen to T cells.

 CD1 presentation is particularly important in the


activation of gamma/delta T cells but there is evidence
that alpha/beta T cells can also be activated in this
manner

09/15/2025 Advanced Immunology 44


T cells development

 For a T cell to be functional it needs to have a functional


T cell receptor on its surface.

 That T cell receptor must be able to recognize antigen as


a peptide in the groove of self MHC.

 The T cell receptor must be appropriately linked to


CD3 for proper signal transduction.

 Finally, you don’t want the T cell to be self-reactive. The


development of the T cell receptor and the testing of the
reaction of the receptor occur in the thymus.

09/15/2025 Advanced Immunology 45


T cells development...

 Pre-T cells (like pre-B cells) arise from lymphoid


stem cell precursors in the bone marrow.

 They leave the bone marrow and transit, via the


blood stream, to the thymus where they develop
and undergo “thymic education”.

09/15/2025 Advanced Immunology 46


T cells development...

 The first thing to happen is that the β chain of the


TcR undergoes gene rearrangement.

 Just like in surface antibody development in B


cells this β chain in T cells has to be tested for
functional activity before the α chain is
rearranged.

09/15/2025 Advanced Immunology 47


T cells development...

 The β chain is brought to the surface with a


surrogate (pseudo) α chain and tested.

 If it links properly with the signal transduction


components then the T cell goes on to rearrange
α chain genes and put a mature T cell receptor on
its surface.

 At this stage the T cell is both CD4+ and CD8+


(called a double positive cell).

09/15/2025 Advanced Immunology 48


TCR genetics: Similar to Ig genetics

V regions (70-80) J regions (61) C region (1)

 chain

V regions (52) D1 (1) J1 (6) C1 (1) D2 (1) J2 (7) C2 (1)

 chain

09/15/2025 Advanced Immunology 49


T cells development...

 Putting a T cell receptor on the surface is only the first


stage.

 T cells are very specific in that not only do they have to


recognize specific antigen, they have to recognize it in
a specific context, as a peptide in the groove of self MHC.

 Therefore, the next step is to make sure the T cell can


recognize self MHC.

 The T cells interact with MHC bearing cells in the thymus


and if the TcR binds weakly to them the cell goes on to
further development

09/15/2025 Advanced Immunology 50


T cells development...

 If the T cell gives the MHC bearing cells the cold shoulder
then it does not receive the life prolonging signals that
come from this interaction and it dies.

 Most of the T cells in the thymus will fail this test and die.

 If they pass this test they then have to be tested for self
reactivity.

 If they interact with MHC bearing cells very strongly,


indicating that the self peptide and the self MHC is their
chosen antigen to respond to, then they will be killed.

09/15/2025 Advanced Immunology 51


T cells development...

 If they pass both of these tests they will become mature


double positive T cells.

 These cells will then lose either CD4 or CD8 to become naive
CD4+ cells or CD8+ cells.

 The cells with a CD4 marker are called helper T cells (Th
cells).

 The CD8 positive cells that develop are cytotoxic T cells (Tc
cells).

 Th and Tc cells both have a TcR, but they perform very


different functions in the immune system.
09/15/2025 Advanced Immunology 52
Th cell activation

 T cell activation is, in general, similar for both Th and Tc


cells but each are considered separately for clarity.

 Th cell activation is initiated by the interaction of TcR-


CD3 complex with antigen-MHC class II molecules on
the surface of an antigen presenting cell.

 This interaction initiates a cascade of biochemical


events in the T cell that eventually results in growth and
proliferation of the T cell.

 This occurs primarily through an increase in IL-2


secretion by the T cell and an increase in IL-2 receptors
on the T cell [Link] Immunology
09/15/2025 53
Th cell activation...

 IL-2 is a potent T cell growth cytokine which, in T cell


activation, acts in an autocrine (self-stimulating) fashion to
promote the growth, clonal proliferation and differentiation
of the T cell recently stimulated by antigen.

 The T cell receptor is an antigen recognition molecule and


therefore the T cell that best responds to the antigen presented
is the one that gets turned on.

 All its progeny will recognize the same “T cell epitope” of the
original antigen, since they will all have identical T cell
receptors.

 Naive, bystander T cells have no IL-2 receptors and thus cannot


be involved.
09/15/2025 Advanced Immunology 54
Th cell activation...

 The activation takes place through the T cell


receptor complex and is aided by the CD4 molecule
on Th cells as well as other accessory molecules,
such as CD45, CD28 and CD2.

 The APC also provides a co-stimulatory signal


through molecules such as B7 and by the secretion
of co-stimulatory cytokines (such as IL-1 or IL-12).

 Activated Th cells then continue to become effector


cells whose role includes B cell help and activation
of effector cells such as macrophages.
09/15/2025 Advanced Immunology 55
Antigen Presenting Cells (APC)

 Antigen presenting cells are a functionally defined


group of cells which are able to take up antigens
and present them to T lymphocytes in a
recognizable form (in the groove of an MHC class
II molecule).

 Although many cells can do this, the cells which


are most efficient, the so-called "professional
antigen presenting cells", are dendritic cells.

09/15/2025 Advanced Immunology 56


Antigen Presenting Cells (APC)...

 These are professional cells because they are


highly effective at producing the "second signal"
required for naive T cell activation.

 The APC first internalizes the antigen (maybe in


the form of a bacteria or bacterial product),
processes it (breaks it down into antigenic
peptides by digesting it with enzymes
(lysosyme)and then expresses the antigen
fragment (peptide) on its surface in the groove of
an MHC class II molecule. Now the antigen is in
the form recognizable by T cells
09/15/2025 Advanced Immunology 57
Antigen Presenting Cells (APC)...

 Antigen presentation by APC is a required first


step in Th cell activation.

 Presentation to naive T cells like this happens


almost exclusively in the lymph nodes and
spleen.

 This means the dendritic cells have to leave the


site of infection and travel to the local draining
node to accomplish this.

09/15/2025 Advanced Immunology 58


Antigen Presenting Cells (APC)...

 Dendritic cells are the only cells that can present


antigen to naive T cells but macrophages and B
cells can present antigen to activated or memory
T cells.

 It is thought that antigen presentation to memory


T cells by macrophages is very important the
second time you see an infection since this can
happen at the site of infection

09/15/2025 Advanced Immunology 59


T Cell Help -Th Cells

 The generation of an effective immune response


depends on the activation of Th cells.

 Both humoral and cell-mediated responses are


initiated or amplified by Th cells. The importance
of these CD4+ cells to a functioning immune
system is clearly demonstrated by the impact of
the HIV virus.

 This virus preferentially kills CD4+ T cells and the


devastating immune deficiency syndrome resulting
from this is the disease known as AIDS.
09/15/2025 Advanced Immunology 60
T Cell Help -Th Cells...

 Mature B cells that have already seen antigen


require contact with a T cell in order to become
plasma cells or memory cells (T-B cell
interaction).

 The T cells provide signals to the B cell through


contact of the TcR complex with processed
antigen presented on Class II MHC by the B cell.

09/15/2025 Advanced Immunology 61


T Cell Help -Th Cells...

 In addition, the activated T cell produces


cytokines which stimulate B cell proliferation and
the differentiation of these cells into antibody
secreting plasma cells.

 Th cell cytokines are required for “class switch”


so that isotypes other than IgM can be produced.

09/15/2025 Advanced Immunology 62


T Helper Cell Subsets

 Our understanding of how and why T cells behave


in an immune response has markedly increased
with the discovery that there are at least two
subsets of T helper cells.

 The two subsets look the same and have the same
T cell markers and receptors. However, they
secrete very different cytokines upon activation.

 In addition to other cytokines, the Th1 subset


produces abundant IL-2 and IFN gamma.

09/15/2025 Advanced Immunology 63


T Helper Cell Subsets...

 These cytokines are particularly important in cell-


mediated immunity.

 The Th2 subset is very good at providing B cell help


by secreting abundant IL- 4.

 Because the various cytokines have different effects


in an immune response, sometimes activation of one
subset may be preferable to the other.

 For example, virus infection is best combated with


cytotoxic T cells

09/15/2025 Advanced Immunology 64


T Helper Cell Subsets...

 This response is more likely to be stimulated by


Th1 cytokines.

 Although there is evidence that these subsets are


cross-regulatory (activation of one downregulates
the other) it is likely that they work together in
normal immune responses.

09/15/2025 Advanced Immunology 65


T Helper Cell Subsets...

 To use the example above, viral infection is also


marked by anti-viral antibody.

 There are, however, some instances where only


Th1 immunity is useful, as in the case of
tuberculosis infection, where few antibodies are
made to the pathogen and they have no role in
dealing with the infection.

09/15/2025 Advanced Immunology 66


Reading assignment
Read about

 Regulation of the complement activity

 Disorders of the complement system

 Differentiate among the classical, alternate and


Lectin binding path ways

09/15/2025 Advanced Immunology 67


Next class will be
 effecter cells of
the immune sytem

09/15/2025 Advanced Immunology 68

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