??? ????(complement system)

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??? ????(complement system)
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? ? ?. ????? ?. ????? ?. ??????? ?????? ??????
???? ???????
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??? ?????(30????)
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1.????????? Classical pathway C1 (C1q, C1r,C1s),
C4, C2, C3 MBLectin Pathway (mannan-binding
lectin,?????????) MBL, MASP-1 (serine
protease,??????-1),MASP-2, C4, C2, C3
Alterative pathway Factor B, Factor D,
C3 Terminal pathway C5, C6, C7, C8, C9
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2.??????????? ?????C1INH(C1 inhibitor,C1???),Fact
or I,Factor H, properdin(???), C4bp(C4 binding
protein, C4????), S??(Sp/Vn),Sp40/40 ???CR1,
MCP (membrane co-factor protein, CD46,?????),
DAF(decay-accelerating factor, ??????),MIRL
(membrane inhibitor of reactive lysis,CD59,
??????????), ?????? 3.????(???) CR1, CR2, CR3,
CR4, CR5, C1qR, C3aR, C5aR
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• ????????????
• ??????????????????,?C3a?C3b?,??????????a,????b,?C2
  ??,????C2a,????C2b
• ??????????????????????
• ?????????????????i,?iC3b

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• ???
• ?????

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??????,???????????????????????????????????,
???????????? ?????????,?????????????,???????????
???,??????????
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??????? 1. ????,??????,???????????
?????3???????3???
????
????
MBLectin??
??????
????
?????
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?????????(classical pathway) C1(C1q?C1r?C1s)?C4?C
2?C3?C5?C6?C7?C8?C9 ???? ????
?????
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???? ????????,????????,?Fc??????????,??C1???????
?,??????????????????
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(No Transcript)
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C1???C1q?C1r?C1s????????????Ca2???
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C1q????,???,??????????C1q?Ig??????C1r?C1s?C
1q?????????C1q?????????IgM?IgG Fc??????,C1q???????
??????,??C1r???,????????????C1r,????C1s??????,????
???????????,?????C4?C2?
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• ????C1q??(??,??,????,????,?????)???????????,??????
  ???(?????)???Ig???????,Ig??Fc????????,IgM?CH3??IgG
  1?IgG2?IgG3?CH2??C1q ??????????,C1q???????????
• ?C1q?????????????????????C1q?????IgM??????,???Fc?,
  ???IgM??????C1q??IgG???,?????????IgG?????,???C1q??
  ?

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(No Transcript)
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?????????
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• C1s???C4,???????C4a?????,????C4b???????-?????????M
  g2?????,C2?????C4b???????,???C1s??,???????C2a????
  ??,????C2b??C4b??C4b2b???,????????C3????
• C4b2b??C4b??C3??,C2b???C3,?????????????,??????????
  10???C3b?????????C4b2b??,??C4b2b3b???,????????C5
  ????

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???????????? thioester bond(???) hydroxyl or
amino group
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(No Transcript)
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???????????????(MBLectin pathway) MBL?MASP?C4?C2?
C3?C5?C6?C7?C8?C9 ???? ????
????? Mannose-binding lectin, MBL-associated
serine protease
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MBL?C????,??????,??????????,?????????????????,?
?????? ?????????????lectin(ficolin,???L-ficolin?
H-ficolin?M-ficolin,????ficolin-A),????????
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MBL??????????,???????,???????,MBL?1??????????
????????????,????????????TNF-??IL-1?IL-6,?????????
?MBL?C?????????????????????? MBL?C1q????????????
??,???????????
MBL??????
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MBL?????????????,???????????,??MBL?????????(MBL
-associated serine protease,MASP)?
MASP-2???C2?C4,MASP-1?????C3?MASP-3?????,???????MB
L??,???MBL????????
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MASP1/3?MASP2/sMAP?????
MASP ??????????,???????MBL-MASP?????MBL??????,??MA
SP,MASP?????????,?????????
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(No Transcript)
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• ??????????
• (alternative pathway)

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??????
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????????????????????????????IgA?IgG4??????????,?
????C1q???,?????????????????????????????????????
??????????????????????,????????????????????
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C3??????????????????????????????????C3b??B?????
??D??????????B????????Ba????Bb?Ba?????,Bb????C3b,?
???C3bBb??????????C3???,??Bb?????????,???C3?
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????C3?????C3??C3a?C3b,???????????C3bBb????C3bBb
3b,???????????C5???,?????????C5???C4b2b3b??,????C5
,??????????
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?????????2??? C3bBb????,????????????????C3bBb,????
??
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(No Transcript)
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(No Transcript)
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??????????????????? 1.????????????????,???C3b?????
??????,?????C3???? 2.?????????????
?????,??????????C3b,??C3b???????????????????,C3b??
????(????????)??,??????????,????????????????,?C3b?
?B???????C3bB,??????????C3bBb?
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?????????????
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MAC(membrane attack complex)???
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???????C5b8???,??1016(1219)?C9?????C5b9
, ?MAC.???????C9?????????,??????C9(poly-C9)???????
????,???????10nm????
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(No Transcript)
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??? ???????
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• ?????
• ????????
• ??????????????, ??????????????????,????????????

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• ????
• ???????
• ???????
• ????????

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????(???????) ???????????????,?
C3b,C4b???60?? C4b2b?????37ºC?5??
C5b????37ºC?2.3?? C567???0.1? C5a,
C3a, C4a????????
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?????????

• ???????

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a. C1???(C1 inhibitor, C1INH)C1INH?????C1r?C1s???
??????????,?C1r?C1s??????????????,
C1INH???????IC???C1?????,??????C1????? b.
??????C3?????? a) C4????( C4binding protein,
C4bp)?????1(complement receptor 1,CR1)
C4bp??????,CR1????,?????C4b??,?????C4b?C2??,?????
???C3????C4b2b???,?????????,C4b?CR1????????,??I???
C4b????????
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b) I??I?????????????,??C4b???C4c?C4d????????,
??????????,??C3??????I?????C3b? c)
?????(membrane cofactor protein, MCP)
MCP???????????????????,???????,??I?????C4b??,?????
??C4b2b??? d) ??????(decay-accelerating factor,
DAF)DAF(?CD55)??????????????????????????,??C2???
C4b???,????C3??????,???????
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2. ???????
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• ??????C3?????????H????B???Bb??????C3b,???C3b?I??
  ???????,CR1?DAF???????B???C3b???I????C3b???????iC3
  bH???CR1?MCP????????,??I????C3b???MCP?CR1?????C3
  b?H???????
• ??????C3?????CR1?DAF???Bb?????????C3???????
• ???????????????(properdin, P??)?C3bBb?????????,?
  ?C3bBb?????10?,????C3?????C3????

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3. ???????????

• CD59??C7?C8?C5b?6?????,????MAC??
• ??????(homologous restriction factor,
  HRF)???C8????(C8-binding protein)???C9?C8???

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(No Transcript)
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??? ????

• ????1(CR1)????????,?C3b?C4b??????
• ????2(CR2)????????
• ????3(CR3)iC3bR?Mac-1?CD11b/CD18
• ????4(CR4)P150.95?CD11c/CD18

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

• ????????????????????,???????
• ?????????????????????????,?C3a?C4a?C5a?,??????

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(No Transcript)
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(No Transcript)
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(No Transcript)
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???????????

• ?????????
• ??????

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3????????
59
?????????
C1q?MBL?C3b????????????
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??????????????
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??? ???????

• ???????????
• ????????

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• ????????????
• ?????????????(??????????????????????????)
• ????????(?????????)
• ????(???????)??????

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• ?????????????,?56?C??30?????,?????????,?0-10?C??
  ?????3-4?,????????-20 ?C??????????????????????????
  ???

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The End