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

Description: Active  
Expression host: Human Cells  
10154-H08H-20
10154-H08H-10
20 µg 
10 µg 
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Expression host: Human Cells  
10154-H02H-20
10154-H02H-10
20 µg 
10 µg 
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Expression host: Human Cells  
51140-M08H-20
51140-M08H-10
20 µg 
10 µg 
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Complement Component C2 Related Area

Complement Component C2 Related Pathways

Complement Component C2 Related Protein, Antibody, cDNA Gene, and ELISA Kits

Complement Component C2 Related Protein, Antibody, cDNA Gene, and ELISA Kits

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Complement Component C2 Summary & Protein Information

Complement Component C2 Background

Catalytic activity: 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.
Subunit structure: 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}.
Domain: The MIDAS-like motif in the VWFA domain binds divalent metal cations.
Subcellular location: Secreted.
Involvement in disease: 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.
Sequence similarity: 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 Alternative Name

CO2,ARMD14, [homo-sapiens]
ARMD14, [Human]
C2,RP24-317H19.3, [mouse]

Complement Component C2 Related Studies

  • 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.
  • Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"