보충 C2 는 인간에게 C2 유전자 에 의해 암호화된 단백질 이다.[5] 이 유전자에 의해 암호화된 단백질은 복수 도메인 세린 프로테아제 역할을 하는 보완 시스템 의 고전적인 경로 의 일부분이다. C2의 결핍은 특정 자가면역질환과 관련이 있다.[5]
함수 보완 활성화 의 고전적 경로와 강의식 경로에서 C3-변환제와 C5-변환제의 형성 은2+ Mg가 존재하는 곳에서 활성화된 표면 결합 C4b에 C2의 결합을 요구한다. 결과 C4bC2 복합체는 C1s 또는 MASP2 에 의해 C2a와 C2b로 분할된다.C4b에 묶인 상태에서 C1s에 의한 C2의 갈라짐은 C2b의 순응적인 회전을 초래하는 반면, 공개된 C2a 파편은 원래 구조의 대부분을 유지할 수 있다고 생각된다.
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). 더 작은 파편인 C2a(또는 선원에 따라 C2b)는 유체 단계로 방출된다.[6]
참조 ^ 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: 앙상블 릴리스 89: ENSMUSG000024371 - 앙상블 , 2017년 5월^ "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 .
추가 읽기 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 . 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 . 외부 링크
PDB 갤러리
2odp : C3의 촉매 파편인 구성 요소 C2a와 인간 보어의 C5 변환효소 보완
2odq : C3의 촉매 파편인 구성 요소 C2a와 인간 보완물의 C5 변환제 보완
