Complement component 2

Complement component 2
PDB rendering based on 2i6q.
Available structures PDB 2I6Q, 2I6S, 2ODP, 2ODQ, 3ERB
Identifiers
Symbols	C2; CO2; DKFZp779M0311
External IDs	OMIM: 613927 MGI: 88226 HomoloGene: 45 GeneCards: C2 Gene
EC number	3.4.21.43
Gene Ontology Molecular function • serine-type endopeptidase activity • peptidase activity Cellular component • extracellular region • extracellular region • extracellular space Biological process • proteolysis • complement activation • complement activation, classical pathway • innate immune response Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	717	12263
Ensembl	ENSG00000166278	ENSMUSG00000024371
UniProt	P06681	O70350
RefSeq (mRNA)	NM_000063.4	NM_013484.2
RefSeq (protein)	NP_000054.2	NP_038512.2
Location (UCSC)	Chr c6_COX: 32.03 – 32.05 Mb	Chr 17: 34.99 – 35.03 Mb
PubMed search	[1]	[2]

Complement C2 is a protein that in humans is encoded by the C2 gene.^[1] The protein encoded by this gene is part of the classical pathway of complement system.

Activated C1 cleaves C2 into C2a and C2b. C2a leads to activation of C3. Deficiency of C2 has been reported to associated with certain autoimmune diseases.^[1]

References

1. ^ ^{a b} "Entrez Gene: C2 complement component 2". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=717. 

Further reading

• Bartholomew WR, Shanahan TC (1991). "Complement components and receptors: deficiencies and disease associations". Immunol. Ser. 52: 33–51. PMID 2091785. 
• Campbell RD (1988). "The molecular genetics and polymorphism of C2 and factor B". Br. Med. Bull. 43 (1): 37–49. PMID 3315100. 
• Yu CY (1999). "Molecular genetics of the human MHC complement gene cluster". Exp. Clin. Immunogenet. 15 (4): 213–30. doi:10.1159/000019075. PMID 10072631. 
• Lutsenko SM, Kharchenko VG, Bachurin VI, Lomakin MM (1976). "[Circulating blood volume and regional hemodynamics in acute gastrointestinal hemorrhage]". Sovetskaia meditsina (2): 38–41. PMID 1084023. 
• Zhu ZB, Hsieh SL, Bentley DR et al. (1992). "A variable number of tandem repeats locus within the human complement C2 gene is associated with a retroposon derived from a human endogenous retrovirus". J. Exp. Med. 175 (6): 1783–7. doi:10.1084/jem.175.6.1783. PMC 2119228. PMID 1350302. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2119228. 
• Lappin DF, Guc D, Hill A et al. (1992). "Effect of interferon-gamma on complement gene expression in different cell types". Biochem. J. 281 ( Pt 2) (Pt 2): 437–42. PMC 1130704. PMID 1531292. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1130704. 
• Johnson CA, Densen P, Hurford RK et al. (1992). "Type I human complement C2 deficiency. A 28-base pair gene deletion causes skipping of exon 6 during RNA splicing". J. Biol. Chem. 267 (13): 9347–53. PMID 1577763. 
• Lappin DF, Birnie GD, Whaley K (1991). "Interferon-mediated transcriptional and post-transcriptional modulation of complement gene expression in human monocytes". Eur. J. Biochem. 194 (1): 177–84. doi:10.1111/j.1432-1033.1990.tb19443.x. PMID 1701385. 
• Horiuchi T, Macon KJ, Kidd VJ, Volanakis JE (1989). "cDNA cloning and expression of human complement component C2". J. Immunol. 142 (6): 2105–11. PMID 2493504. 
• Cole FS, Whitehead AS, Auerbach HS et al. (1985). "The molecular basis for genetic deficiency of the second component of human complement". N. Engl. J. Med. 313 (1): 11–6. doi:10.1056/NEJM198507043130103. PMID 2582254. 
• Bentley DR (1987). "Primary structure of human complement component C2. Homology to two unrelated protein families". Biochem. J. 239 (2): 339–45. PMC 1147286. PMID 2949737. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1147286. 
• Bentley DR, Campbell RD, Cross SJ (1985). "DNA polymorphism of the C2 locus". Immunogenetics 22 (4): 377–90. doi:10.1007/BF00430921. PMID 2997031. 
• Kam CM, McRae BJ, Harper JW et al. (1987). "Human complement proteins D, C2, and B. Active site mapping with peptide thioester substrates". J. Biol. Chem. 262 (8): 3444–51. PMID 3546307. 
• Wu LC, Morley BJ, Campbell RD (1987). "Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements". Cell 48 (2): 331–42. doi:10.1016/0092-8674(87)90436-3. PMID 3643061. 
• Gagnon J (1984). "Structure and activation of complement components C2 and factor B". Philos. Trans. R. Soc. Lond., B, Biol. Sci. 306 (1129): 301–9. doi:10.1098/rstb.1984.0091. PMID 6149575. 
• Bentley DR, Porter RR (1984). "Isolation of cDNA clones for human complement component C2". Proc. Natl. Acad. Sci. U.S.A. 81 (4): 1212–5. doi:10.1073/pnas.81.4.1212. PMC 344796. PMID 6199794. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=344796. 
• Parkes C, Gagnon J, Kerr MA (1983). "The reaction of iodine and thiol-blocking reagents with human complement components C2 and factor B. Purification and N-terminal amino acid sequence of a peptide from C2a containing a free thiol group". Biochem. J. 213 (1): 201–9. PMC 1152109. PMID 6555044. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1152109. 
• Kerr MA, Gagnon J (1982). "The purification and properties of the second component of guinea-pig complement". Biochem. J. 205 (1): 59–67. PMC 1158446. PMID 6922702. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1158446. 

External links

• MeSH Complement+2

Inhibitors

CLA: C1-inhibitor - Decay-accelerating factor/CD59 - Factor I

CL: C4BP

A: Factor H

Complement receptors

CR1 - CR2 - CR3 - CR4 - CD11b/CD11c/CD18 - Anaphylatoxin (C3a, C5a)

M: LMC cell/phys/auag/auab/comp, igrc imdf/ipig/hyps/tumr proc, drug(L3/4)