????????????;??????????MBL??????????;????????

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• ??????????????????????MBL??????????????????
• ???????MBL????????????
• ????????????????

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Belgian bacteriologist and immunologist who
received the Nobel Prize for Physiology or
Medicine in 1919 for his discovery of immunity
factors in blood serum this was a development
vital to the diagnosis and treatment of many
dangerous contagious diseases.
Jules Bordet  (1870-1961), discoverer of
complement
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• ???????
• ???????

???? ????C1????I???C4?????H???
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??? ?????

• Pathway of Complement Activation

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• (?)????
• Classical Pathway of Complement
  Activation
• (?)????
• Alternative Pathway of Complement
  Activation
• (?)??????????
• MBLPathway of Complement Activation
• (?)????
• Terminal Pathway of Complement
  Activation

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?????????

• Classical Pathway of Complement Activation

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????????

• Ag-Ab?????????????????
• ????
• 1. C1?IgM?CH3??IgG?????CH2????????
• 2.???C1????????Ig???Fc????
• 3.?????????????Fc????C1q???

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IgG?????????????
IgM CH3?,IgG CH2?
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? ? ? ? ? ? ? ? ?
IgM/IgG ???
C1q r s
Ca
Ca
Mg
C4bC2(C4b2)
C4
C4b2b
(C3???)
C4a
C4b2b3b
C2a
(C5???)
C3
C3b
C3a
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?????MBL??

• MBL Pathway of Complement Activation

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????????(MBL)???MBL?????????(MASP)??,?C4?C
2?C3??????,????C5????
?????????

• ????
• 1? MASP??
• 2?C3???(C4b2b)??
• 3?C5???(C4b2b3b)??

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C4
C4a C4b
????
????
MASP
C4b2b
MBL
MBL
(C3???)
MASP
C2
C2a C2b
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???????????
?????C3????,?B???D??????,????C5????
?????????(???,????)

• ????
• 1?C3??
• 2?C3???(C3bBb)??
• 3?C5???(C3bnBb)??
• 4?C3b?????

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?????????

• Alternative Pathway of Complement Activation

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???????????

• Terminal Pathway of Complement Activation

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• ?C5?????C5??C5a?C5b??,?C6?C7?C8?C9?????,??
  ??C5b6789???????????(MAC),???????

???? 1?C5b?? 2?C5b678??
3?C5b6789(MAC)??
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C5bC6C7C8C9 MACs
?????????
C7
C6
C8
10-17
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?????? ??????C3b5b?C6?C7?C8??????C5b678??????
????C9????????,??????????MACs,??????????
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??? ???????

• Regulation of Complement Activation

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• ?????????
• ???????????
• ???????
• ???????
• ???????????

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???????

• C1????
• ??????C3?????
• I??
• ?????
• ??????

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• Factor H (H??)removes Bb from the alternative
  pathway C3 convertase(C3 ???) breaking the
  positive feedback loop.
• Factor I (I??)inactivates C3b and C4b.
• C1 inhibitor (C1INH, C1???) binds to sites on
  activated C1r and C1s shutting down (??) their
  proteolytic activity.

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???????

• ??????C3??????
• ??????C3?????

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???????????
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??? ????????

• MAC???????????
• ??????????????
• 1.????
• 2.????????
• 3.??????
• 4.??????

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??? ????????

• Function of Complement Activation

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?????????????

• ??????????
• ????
• ??????

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? ? ? ? ? ? ?
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???????????

• ???????
• ??????

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?????????

• ???????????
• ?????????????
• ?????????????
• ????????

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? ?
1????????????????????????????????????????????????
????,???????????????????? ????????????,????,????
? 2????????????,????,MBP??,
3??????????C1INH, I ?? H
???DAF???????????? 4?????CR1,CR2,CR3/CR4,CR5a?
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• ? ?
• ??????(??)?MAC??????
• ??1?????????????????
• 2?????????????????
• 3??????????

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