Complement component 2

From Wikipedia, the free encyclopedia

Complement component 2

PDB rendering based on 2i6q.

Available structures

Ortholog search: PDBe, RCSB

List of PDB id codes
2I6S, 3ERB

Identifiers

C2; CO2

External IDs

OMIM: 613927 MGI: 88226 HomoloGene: 45 GeneCards: C2 Gene

Gene Ontology
Molecular function	• serine-type endopeptidase activity
Cellular component	• extracellular region • extracellular space
Biological process	• proteolysis • complement activation • complement activation, classical pathway • regulation of complement activation • innate immune response
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

ENSG00000166278

ENSMUSG00000024371

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 6:
31.87 – 31.91 Mb

Chr 17:
34.86 – 34.9 Mb

PubMed search

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.

In the "classical" pathway of complement activation, activated C1 cleaves C4a and C4b, and cleaves C2 into C2a and C2b. The complex of the large fragments of C2 and C4, called C4b2a, is the classical C3 convertase and can activate C3. Deficiency of C2 has been associated with certain autoimmune diseases.^[1]

References

↑ 1.0 1.1 "Entrez Gene: C2 complement component 2".

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.
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.
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.
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.
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.
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.

PDB gallery

2i6q: Complement component C2a

2i6s: Complement component C2a

2odp: Complement component C2a, the catalytic fragment of C3- and C5-convertase of human complement

2odq: Complement component C2a, the catalytic fragment of C3- and C5-convertase of human complement

External links

Complement 2 at the US National Library of Medicine Medical Subject Headings (MeSH)

Proteins: complement system (C, L, A)

Activators/enzymes

Early	C: C1Q/C1R/C1S C4 C4a C2 L: MASP1/MASP2 MBL A: Factor B Factor D Factor P/Properdin

Middle	C3 C3a C3b/iC3b C5 C5a C3-convertase C5-convertase

Late	MAC C6 C7 C8 C9

Inhibitors

CL: C4BP

A: Factor H

Complement receptors

M: LMC

cell/phys/auag/auab/comp, igrc

imdf/ipig/hyps/tumr

proc, drug (L3/4)

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