Complement C2 is a protein that in humans is encoded by the C2gene.[5] The protein encoded by this gene is part of the classical pathway of the complement system, acting as a multi-domain serine protease. Deficiency of C2 has been associated with certain autoimmune diseases.[5]
In the classical and lectin pathways of complement activation, formation of the C3-convertase and C5-convertases requires binding of C2 to an activated surface-bound C4b in the presence of Mg2+; the resultant C4bC2 complex is cleaved by C1s or MASP2 into C2a and C2b. It is thought that cleavage of C2 by C1s, while bound to C4b, results in a conformational rotation of C2b whereas the released C2a fragment may retain most of its original structure.
C2b is the smallest , enzymatically active, fragment of C3 convertase in this pathway, C4b2b (NB: some sources now refer to the larger fragment of C2 as C2b, making the C3 convertase C4b2b, whereas older sources refer to the larger fragment of C2 as C2a, making the C3 convertase C4b2a). The smaller fragment, C2a (or C2b, depending on the source) is released into the fluid phase.[6]
^Krishnan V, Xu Y, Macon K, Volanakis JE, Narayana SV (2009). "The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation". Acta Crystallographica D. 65 (Pt 3): 266–274. doi:10.1107/S0907444909000389. PMC2651757. PMID 19237749.
Further reading
Bartholomew WR, Shanahan TC (1991). "Complement components and receptors: deficiencies and disease associations". Immunology Series. 52: 33–51. PMID 2091785.
Campbell RD (Jan 1987). "The molecular genetics and polymorphism of C2 and factor B". British Medical Bulletin. 43 (1): 37–49. doi:10.1093/oxfordjournals.bmb.a072175. PMID 3315100.
Yu CY (1999). "Molecular genetics of the human MHC complement gene cluster". Experimental and Clinical Immunogenetics. 15 (4): 213–230. doi:10.1159/000019075. PMID 10072631. S2CID 25061446.
Lutsenko SM, Kharchenko VG, Bachurin VI, Lomakin MM (Feb 1976). "[Circulating blood volume and regional hemodynamics in acute gastrointestinal hemorrhage]". Sovetskaia Meditsina (2): 38–41. PMID 1084023.
Zhu ZB, Hsieh SL, Bentley DR, Campbell RD, Volanakis JE (Jun 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". The Journal of Experimental Medicine. 175 (6): 1783–1787. doi:10.1084/jem.175.6.1783. PMC2119228. PMID 1350302.
Lappin DF, Guc D, Hill A, McShane T, Whaley K (Jan 1992). "Effect of interferon-gamma on complement gene expression in different cell types". The Biochemical Journal. 281 (Pt 2): 437–442. doi:10.1042/bj2810437. PMC1130704. PMID 1531292.
Johnson CA, Densen P, Hurford RK, Colten HR, Wetsel RA (May 1992). "Type I human complement C2 deficiency. A 28-base pair gene deletion causes skipping of exon 6 during RNA splicing". The Journal of Biological Chemistry. 267 (13): 9347–9353. doi:10.1016/S0021-9258(19)50430-6. PMID 1577763.
Lappin DF, Birnie GD, Whaley K (Nov 1990). "Interferon-mediated transcriptional and post-transcriptional modulation of complement gene expression in human monocytes". European Journal of Biochemistry. 194 (1): 177–184. doi:10.1111/j.1432-1033.1990.tb19443.x. PMID 1701385.
Horiuchi T, Macon KJ, Kidd VJ, Volanakis JE (Mar 1989). "cDNA cloning and expression of human complement component C2". Journal of Immunology. 142 (6): 2105–2111. PMID 2493504.
Cole FS, Whitehead AS, Auerbach HS, Lint T, Zeitz HJ, Kilbridge P, Colten HR (Jul 1985). "The molecular basis for genetic deficiency of the second component of human complement". The New England Journal of Medicine. 313 (1): 11–16. doi:10.1056/NEJM198507043130103. PMID 2582254.
Bentley DR (Oct 1986). "Primary structure of human complement component C2. Homology to two unrelated protein families". The Biochemical Journal. 239 (2): 339–345. doi:10.1042/bj2390339. PMC1147286. PMID 2949737.
Kam CM, McRae BJ, Harper JW, Niemann MA, Volanakis JE, Powers JC (Mar 1987). "Human complement proteins D, C2, and B. Active site mapping with peptide thioester substrates". The Journal of Biological Chemistry. 262 (8): 3444–3451. doi:10.1016/S0021-9258(18)61371-7. PMID 3546307.
Wu LC, Morley BJ, Campbell RD (Jan 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): 331–342. doi:10.1016/0092-8674(87)90436-3. PMID 3643061. S2CID 32752642.
Gagnon J (Sep 1984). "Structure and activation of complement components C2 and factor B". Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 306 (1129): 301–309. Bibcode:1984RSPTB.306..301G. doi:10.1098/rstb.1984.0091. PMID 6149575.
Bentley DR, Porter RR (Feb 1984). "Isolation of cDNA clones for human complement component C2". Proceedings of the National Academy of Sciences of the United States of America. 81 (4): 1212–1215. Bibcode:1984PNAS...81.1212B. doi:10.1073/pnas.81.4.1212. PMC344796. PMID 6199794.
Parkes C, Gagnon J, Kerr MA (Jul 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". The Biochemical Journal. 213 (1): 201–209. doi:10.1042/bj2130201. PMC1152109. PMID 6555044.
Kerr MA, Gagnon J (Jul 1982). "The purification and properties of the second component of guinea-pig complement". The Biochemical Journal. 205 (1): 59–67. doi:10.1042/bj2050059. PMC1158446. PMID 6922702.
complement, component, complement, protein, that, humans, encoded, gene, protein, encoded, this, gene, part, classical, pathway, complement, system, acting, multi, domain, serine, protease, deficiency, been, associated, with, certain, autoimmune, diseases, c2a. Complement C2 is a protein that in humans is encoded by the C2 gene 5 The protein encoded by this gene is part of the classical pathway of the complement system acting as a multi domain serine protease Deficiency of C2 has been associated with certain autoimmune diseases 5 C2Available structuresPDBOrtholog search PDBe RCSBList of PDB id codes2I6S 3ERB s2ODQIdentifiersAliasesC2 ARMD14 CO2 complement component 2 complement C2External IDsOMIM 613927 MGI 88226 HomoloGene 45 GeneCards C2Gene location Human Chr Chromosome 6 human 1 Band6p21 33Start31 897 785 bp 1 End31 945 673 bp 1 Gene location Mouse Chr Chromosome 17 mouse 2 Band17 B1 17 18 41 cMStart35 081 580 bp 2 End35 117 241 bp 2 RNA expression patternBgeeHumanMouse ortholog Top expressed inliverright lobe of liverplacentaspleenupper lobe of left lunggallbladderappendixright lunglymph nodeduodenumTop expressed inirisadrenal glandwhite adipose tissueleft lobe of liversciatic nerveileumciliary bodysubcutaneous adipose tissuecarotid bodyfallopian tubeMore reference expression dataBioGPSMore reference expression dataGene ontologyMolecular functionpeptidase activity serine type peptidase activity hydrolase activity metal ion binding serine type endopeptidase activity protein bindingCellular componentextracellular region extracellular exosome extracellular spaceBiological processcomplement activation positive regulation of apoptotic cell clearance regulation of complement activation response to nutrient proteolysis complement activation classical pathway immune system process innate immune responseSources Amigo QuickGOOrthologsSpeciesHumanMouseEntrez71712263EnsemblENSG00000206372ENSG00000235017ENSG00000235696ENSG00000226560ENSG00000204364ENSG00000166278ENSG00000231543ENSMUSG00000024371UniProtP06681Q5JP69P21180RefSeq mRNA NM 000063NM 001145903NM 001178063NM 001282457NM 001282458NM 001282459NM 013484RefSeq protein NP 000054NP 001139375NP 001171534NP 001269386NP 001269387NP 001269388NP 000054 2NP 038512Location UCSC Chr 6 31 9 31 95 MbChr 17 35 08 35 12 MbPubMed search 3 4 WikidataView Edit HumanView Edit Mouse Contents 1 Function 2 References 3 Further reading 4 External linksFunction EditIn the classical and lectin pathways of complement activation formation of the C3 convertase and C5 convertases requires binding of C2 to an activated surface bound C4b in the presence of Mg2 the resultant C4bC2 complex is cleaved by C1s or MASP2 into C2a and C2b It is thought that cleavage of C2 by C1s while bound to C4b results in a conformational rotation of C2b whereas the released C2a fragment may retain most of its original structure C2b is the smallest enzymatically active fragment of C3 convertase in this pathway C4b2b NB some sources now refer to the larger fragment of C2 as C2b making the C3 convertase C4b2b whereas older sources refer to the larger fragment of C2 as C2a making the C3 convertase C4b2a The smaller fragment C2a or C2b depending on the source is released into the fluid phase 6 References Edit a b c ENSG00000235017 ENSG00000235696 ENSG00000226560 ENSG00000204364 ENSG00000166278 ENSG00000231543 GRCh38 Ensembl release 89 ENSG00000206372 ENSG00000235017 ENSG00000235696 ENSG00000226560 ENSG00000204364 ENSG00000166278 ENSG00000231543 Ensembl May 2017 a b c GRCm38 Ensembl release 89 ENSMUSG00000024371 Ensembl May 2017 Human PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Mouse PubMed Reference National Center for Biotechnology Information U S National Library of Medicine a b Entrez Gene C2 complement component 2 Krishnan V Xu Y Macon K Volanakis JE Narayana SV 2009 The structure of C2b a fragment of complement component C2 produced during C3 convertase formation Acta Crystallographica D 65 Pt 3 266 274 doi 10 1107 S0907444909000389 PMC 2651757 PMID 19237749 Further reading EditBartholomew WR Shanahan TC 1991 Complement components and receptors deficiencies and disease associations Immunology Series 52 33 51 PMID 2091785 Campbell RD Jan 1987 The molecular genetics and polymorphism of C2 and factor B British Medical Bulletin 43 1 37 49 doi 10 1093 oxfordjournals bmb a072175 PMID 3315100 Yu CY 1999 Molecular genetics of the human MHC complement gene cluster Experimental and Clinical Immunogenetics 15 4 213 230 doi 10 1159 000019075 PMID 10072631 S2CID 25061446 Lutsenko SM Kharchenko VG Bachurin VI Lomakin MM Feb 1976 Circulating blood volume and regional hemodynamics in acute gastrointestinal hemorrhage Sovetskaia Meditsina 2 38 41 PMID 1084023 Zhu ZB Hsieh SL Bentley DR Campbell RD Volanakis JE Jun 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 The Journal of Experimental Medicine 175 6 1783 1787 doi 10 1084 jem 175 6 1783 PMC 2119228 PMID 1350302 Lappin DF Guc D Hill A McShane T Whaley K Jan 1992 Effect of interferon gamma on complement gene expression in different cell types The Biochemical Journal 281 Pt 2 437 442 doi 10 1042 bj2810437 PMC 1130704 PMID 1531292 Johnson CA Densen P Hurford RK Colten HR Wetsel RA May 1992 Type I human complement C2 deficiency A 28 base pair gene deletion causes skipping of exon 6 during RNA splicing The Journal of Biological Chemistry 267 13 9347 9353 doi 10 1016 S0021 9258 19 50430 6 PMID 1577763 Lappin DF Birnie GD Whaley K Nov 1990 Interferon mediated transcriptional and post transcriptional modulation of complement gene expression in human monocytes European Journal of Biochemistry 194 1 177 184 doi 10 1111 j 1432 1033 1990 tb19443 x PMID 1701385 Horiuchi T Macon KJ Kidd VJ Volanakis JE Mar 1989 cDNA cloning and expression of human complement component C2 Journal of Immunology 142 6 2105 2111 PMID 2493504 Cole FS Whitehead AS Auerbach HS Lint T Zeitz HJ Kilbridge P Colten HR Jul 1985 The molecular basis for genetic deficiency of the second component of human complement The New England Journal of Medicine 313 1 11 16 doi 10 1056 NEJM198507043130103 PMID 2582254 Bentley DR Oct 1986 Primary structure of human complement component C2 Homology to two unrelated protein families The Biochemical Journal 239 2 339 345 doi 10 1042 bj2390339 PMC 1147286 PMID 2949737 Bentley DR Campbell RD Cross SJ 1985 DNA polymorphism of the C2 locus Immunogenetics 22 4 377 390 doi 10 1007 BF00430921 PMID 2997031 S2CID 11934813 Kam CM McRae BJ Harper JW Niemann MA Volanakis JE Powers JC Mar 1987 Human complement proteins D C2 and B Active site mapping with peptide thioester substrates The Journal of Biological Chemistry 262 8 3444 3451 doi 10 1016 S0021 9258 18 61371 7 PMID 3546307 Wu LC Morley BJ Campbell RD Jan 1987 Cell specific expression of the human complement protein factor B gene evidence for the role of two distinct 5 flanking elements Cell 48 2 331 342 doi 10 1016 0092 8674 87 90436 3 PMID 3643061 S2CID 32752642 Gagnon J Sep 1984 Structure and activation of complement components C2 and factor B Philosophical Transactions of the Royal Society of London Series B Biological Sciences 306 1129 301 309 Bibcode 1984RSPTB 306 301G doi 10 1098 rstb 1984 0091 PMID 6149575 Bentley DR Porter RR Feb 1984 Isolation of cDNA clones for human complement component C2 Proceedings of the National Academy of Sciences of the United States of America 81 4 1212 1215 Bibcode 1984PNAS 81 1212B doi 10 1073 pnas 81 4 1212 PMC 344796 PMID 6199794 Parkes C Gagnon J Kerr MA Jul 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 The Biochemical Journal 213 1 201 209 doi 10 1042 bj2130201 PMC 1152109 PMID 6555044 Kerr MA Gagnon J Jul 1982 The purification and properties of the second component of guinea pig complement The Biochemical Journal 205 1 59 67 doi 10 1042 bj2050059 PMC 1158446 PMID 6922702 External links EditComplement 2 at the US National Library of Medicine Medical Subject Headings MeSH This 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