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Complement Component C2  Protein, Antibody, ELISA Kit, cDNA Clone

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表達宿主: Human Cells  
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10154-H08H-20
10154-H08H-10
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表達宿主: Human Cells  
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10154-H02H-20
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表達宿主: Human Cells  
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51140-M08H-20
51140-M08H-10
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Complement Component C2 相关研究领域

Complement Component C2 相關信號通路

Complement Component C2 概述&蛋白信息

Complement Component C2 研究背景

基因概述: Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. Activated C1 cleaves C2 into C2a and C2b. The serine proteinase C2a then combines with complement factor 4b to create the C3 or C5 convertase. Deficiency of C2 has been reported to associated with certain autoimmune diseases and SNPs in this gene have been associated with altered susceptibility to age-related macular degeneration. This gene localizes within the class III region of the MHC on the short arm of chromosome 6. Alternative splicing results in multiple transcript variants encoding distinct isoforms. Additional transcript variants have been described in publications but their full-length sequence has not been determined
General information above from NCBI
催化活性: Selective cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to form C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to form C5a and C5b.
亞單位結構: C2a interacts with Schistosoma haematobium TOR (via N-terminal extracellular domain). This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection. {ECO:0000269|PubMed:10734221}.
結構域: The MIDAS-like motif in the VWFA domain binds divalent metal cations.
亞細胞定位: Secreted.
相關疾病 : DISEASE: Complement component 2 deficiency (C2D) [MIM:217000]: A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent invasive infections. {ECO:0000269|PubMed:8621452, ECO:0000269|PubMed:9670930}. Note=The disease is caused by mutations affecting the gene represented in this entry.
相似的序列: Belongs to the peptidase S1 family. {ECO:0000255|PROSITE-ProRule:PRU00274}.; Contains 1 peptidase S1 domain. {ECO:0000255|PROSITE-ProRule:PRU00274}.; Contains 3 Sushi (CCP/SCR) domains. {ECO:0000255|PROSITE-ProRule:PRU00302}.; Contains 1 VWFA domain. {ECO:0000255|PROSITE-ProRule:PRU00219}.
General information above from UniProt

Complement component C2 is part of the classical complement pathway which plays a major role in innate immunity against infection. C2 is a glycoprotein synthesized in liver hepatocytes and several other cell types in extrahepatic tissues. This pathway is triggered by a multimolecular complex C1, and subsequently the single-chain form of C2 is cleaved into two chains referred to C2a and C2b by activated C1. The second component of complement (C2) is a multi-domain serine protease that provides catalytic activity for the C3 and C5 convertases of the classical and lectin pathways of human complement. C4b and C2 was investigated by surface plasmon resonance. C2a containing a serine protease domain combines with complement component C4b to form the C3 convertase C4b2a which is responsible for C3 activation, and leads to the stimulation of adaptive immune responses via Lectin pathway. C2 bound to C4b is cleaved by classical (C1s) or lectin (MASP2) proteases to produce C4bC2a. C2 has the same serine protease domain as C4bC2a but in an inactive zymogen-like conformation, requiring cofactor-induced conformational change for activity. Deficiency of C2 (C2D) is the most common genetic deficiency of the complement system, and two types of C2D have been recognized in the context of specific MHC haplotypes. C2D in human is reported to increase susceptibility to infection, and is associated with certain autoimmune diseases, such as rheumatological disorders.

Complement Component C2 別稱

CO2,ARMD14, [homo-sapiens]
C2,CO2,DADB-122G4.1,DKFZp779M0311, [human]
C2,RP24-317H19.3, [mouse]

Complement Component C2 相關文獻

  • Laich A, et al. (2002) Complement C4bC2 complex formation: an investigation by surface plasmon resonance. Biochim Biophys Acta. 1544(1-2): 96-112.
  • Halili MA, et al. (2009) Complement component C2, inhibiting a latent serine protease in the classical pathway of complement activation. Biochemistry. 48(35): 8466-72.
  • Krishnan V, et al. (2009) The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation. Acta Crystallogr D Biol Crystallogr. 65(Pt 3): 266-74.
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