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02-06-09, 10:13 PM
The Complement System

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02-06-09, 10:20 PM
The Complement System

http://www.profelis.org/amc/vorlesungen/gifs/MMPE_13IMM_163_02_eps.gif

innate defense functions
trigger inflammation
attract phagocytes
opsonize antigens
cause cell lysis
activate naïve B-lymphocytes
remove immune complexes

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/F13-01.GIF

the classical pathway
antibody-dependent
proteins of the classical pathway
C1
6 molecules of C1q
2 molecules of C1r
2 molecules of C1s

C3
C5

Ag-Ab complex activates C1

animation showing the assembly of C1 during the classical pathway
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/c1act.gif

animation showing the assembly of C3 convertase
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/c3case.gif

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02-06-09, 10:24 PM
animation showing the cleavage of C3 and the formation of C5 convertase
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/c5case.gif

opsonization and phagocytosis [C3b]

CR1 receptors

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/F13-12A.GIF

inflammation [C5a]

the MAC and lysis [C5b6789n]

animation showing the formation of the MAC
http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/F13-05F.GIF




agglutination

Type III hypersensitivity reaction


rheumatoid arthritis
subacute bacterial endocarditis
immune complex glomerulonephritis
systemic lupus erythematosus
Arthus reaction
Farmer’s lung
polyarteritis nodosa


viral neutralization

chemotaxis [C5a]

animation showing the benefits of C5a and C3b

basophil/mast cell activation [C3a, C4a, C5a]

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-07.GIF

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02-06-09, 10:28 PM
the lectin pathway

mannan-binding lectin (MBL)–dependent

proteins of the lectin pathway

MASP1

MASP2

C4 & C2 cleaved

animation of the lectin pathway
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/lectin.gif

the alternative pathway

animation of the alternative pathway and formation of C3 convertase
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/altact.gif

antibody-independent

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-03.GIF

proteins of the alternative pathway

C3

C3b

C3i
B
D
H
I
P (properdin)

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-02.GIF

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02-06-09, 10:30 PM
animation of the formation of C5 convertase.
http://student.ccbcmd.edu/courses/bio141/lecguide/unit4/innate/images/c5alt.gif

large polysaccharides react with factors B and D


C3bB

C3bBb

formation of activation product that activates C3

Bb

C3bBbP

regulation of complement activity
factor H
factor I
C1 inhibitor (C1INH)

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-06.GIF

complement receptors
CR1

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/F13-14.GIF
CR2
CR3
CR4

http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-08.GIF

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02-06-09, 10:32 PM
disorders of the complement system
http://www.profelis.org/amc/vorlesungen/gifs/nbt1342-F3.gif

C3 deficiency
C2 deficiency

immune complex disorders

systemic lupus erythematosus (SLE)

C9 deficiency
C1INH deficiency

hereditary angioneurotic edema (HANE)
decay accelerating factor (DAF) deficiency

paroxysmal nocturnal hemoglobinura (PNH)


http://www.profelis.org/amc/vorlesungen/jpegs/nrmicro1824-f1.jpeg
http://www.profelis.org/amc/vorlesungen/immunologie/CH13-GIF/T13-09.GIF
http://www.profelis.org/amc/vorlesungen/gifs/nbt1342-F2.gif