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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/10/19 04:19:55」(JST)

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1. 補体系の遺伝性疾患 inherited disorders of the complement system
2. 自然免疫系の概要 an overview of the innate immune system
3. 補体経路 complement pathways
4. 補体系の制御因子および受容体 regulators and receptors of the complement system
5. マンノース結合レクチン欠損症 mannose binding lectin deficiency

English Journal

FCN2 inhibits epithelial-mesenchymal transition-induced metastasis of hepatocellular carcinoma via TGF-β/Smad signaling.

Yang G1, Liang Y1, Zheng T1, Song R1, Wang J1, Shi H1, Sun B1, Xie C1, Li Y1, Han J1, Pan S1, Lan Y1, Liu X1, Zhu M1, Wang Y1, Liu L2.
Cancer letters.Cancer Lett.2016 Aug 10;378(2):80-6. doi: 10.1016/j.canlet.2016.05.007. Epub 2016 May 10.
Hepatocellular carcinoma (HCC) is currently still a major cause of cancer-related deaths. Identifying early metastatic biomarkers and therapeutic targets for HCC is of great importance. Emerging evidence suggest that epithelial-mesenchymal transitions (EMTs) play important roles in tumor metastasis
PMID 27177473

The complement system of elasmobranches revealed by liver transcriptome analysis of a hammerhead shark, Sphyrna zygaena.

Goshima M1, Sekiguchi R2, Matsushita M1, Nonaka M3.
Developmental and comparative immunology.Dev Comp Immunol.2016 Aug;61:13-24. doi: 10.1016/j.dci.2016.03.009. Epub 2016 Mar 15.
Comprehensive studies of the complement genes in basal vertebrates have revealed that cyclostomes have apparently primitive complement systems whereas bony fish have well-developed complement systems comparable to those of mammals. Here we have performed liver transcriptome analysis of a hammerhead
PMID 26987526

FCN2 c.772G>T polymorphism is associated with chronic adenoiditis and/or tonsillitis, but not -4 A>G and -602 G>A.

Erkan AN1, Oz I2, Terzi YK3, Aydin E1, Ozkale M4, Babakurban ST1, Koycu A1, Sahin FI3.
International journal of pediatric otorhinolaryngology.Int J Pediatr Otorhinolaryngol.2016 Aug;87:1-4. doi: 10.1016/j.ijporl.2016.05.017. Epub 2016 May 20.
OBJECTIVE: Ficolins are complement activating peptides that play a role in the initial host defense against infectious pathogens. In the present study, we investigated the relationship between single nucleotide polymorphisms (SNPs) in the ficolin 2 gene (FCN2) and chronic adenotonsillitis in pediatr
PMID 27368434

Japanese Journal

Serum L-ficolin levels in patients with systemic lupus erythematosus

Modern rheumatology 22(6), 899-902, 2012-11-01
NAID 10031127245

補体レクチン経路と凝固系の協調作用 : 補体系研究の新展開

日本血栓止血学会誌 = The Journal of Japanese Society on Thrombosis and Hemostasis 22(4), 164-170, 2011-08-01
NAID 10031162941

2P-019 M-フィコリンのC反応性タンパク質結合部位(蛋白質-構造機能相関,第47回日本生物物理学会年会)

生物物理 49(SUPPLEMENT_1), S109, 2009-09-20
NAID 110008101595

Related Pictures

Ficolin-2 inhibitors are present in sera after prolonged storage at −80 °C [PeerJ] Domain and oligomeric structure of mannose-binding lectin (MBL) and | Download 図補体の活性化経路 JapaneseClass.jp Complement C1Q Human FCN3 ELISA Kit | Product No. ABIN1889410 Human FCN1 ELISA Kit | Product No. ABIN415288 Immunodeficiency Associated with FCN3 Mutation and Ficolin-3 Deficiency | NEJM Ficolin-2 inhibitors are present in sera after prolonged storage at −80 °C [PeerJ]

★リンクテーブル★

リンク元	「補体」「フィコリン」「マンノース結合レクチン」

「補体」

　　[★]

英: complement
同: アレキシン alexin
関: 補体活性化経路、補体価

図：IMM.63

pathway	start	first step
classical pathway	antigen-antibody complex	C1q, C1r, C1s, C4, C2
lectin pathway	mannose-binding lectin or ficolin binds carbohydrate on pathogen surface	MBL/ficolin, MASP-2, C4, C2
alternative pathway	pathogen surface	C3, B, D

補体の相互作用 IMM.62

古典的経路 classical pathway

C1qがカスケードの最初となる。3つの方法で補体カスケードがはじまる。

(1)多価陰イオン表面(例えば、グラム陰性細菌のリポテイコ酸)
(2)バクテリアの多糖のホスホコリンに結合(例えば肺炎球菌のC蛋白質)
(3)抗原抗体複合体に結合して自然免疫と獲得免疫のエフェクター機構を結びつける。

レクチン経路 lectin pathway

炭化水素鎖を認識するレクチンがカスケードの最初となる。

1. lectin MBL：mannose-containing carbohydrates
2. ficolin　：N-acetylglucosamine

代替経路 alternative pathway

C3からはじまる。血漿中のC3が病原体の表面で自発的に活性化されることで補体反応がはじまる。

３つの経路は共通してC3 convertaseを生成。C3 convertaseはC3→C3a+C3b
C3a: C3a is a peptide mediator of local inflammation
C3b: C3b binds covalently to the bacterial cell membrane and opsonizes the bacteria

C5 convertaseはC3bにC3 convertaseが結合してできる
C5 converaseはC5→C5a+C5b
C5a: powerful peptide mediator of inflammation
C5b: C5b,C6,C7,C8,C9: membrane-attack complex

C3aとC5a。C4a

炎症部位に血管外に抗体、補体、食細胞を集め、組織液を増やすことで抗原提示細胞がリンパ節に移動するのを促進する(IMM.75)

C5a,C3a,C4a: smooth muscle contraction.(IMM.75)
C5a,C3a: act on the endo thelial cells lining blood vessels to induce adhesion molecules.(IMM.75)
C5a>C3a>C4a: increase in blood flow, increase vascular permeability, increase binding of phagocytes to exdothielial cells.(IMM.76)
C5a: activates mast cells to release mediators, such as histamine and TNF-α. that contribute the inflammatory response(IMM.76).
C5a: acts directly on neutrophils and monocytes and attracting neutrophils and monocytes(IMM.75)
C3a: contributes to the pypotension and edema seen in endotoxic shock
C5a: activated by endotoxin, funcions in neutrophil chemotaxis