Complement component C9

Details

Name
Complement component C9
Synonyms
Not Available
Gene Name
C9
UniProtKB Entry
P02748Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0049816|Complement component C9
MSACRSFAVAICILEISILTAQYTTSYDPELTESSGSASHIDCRMSPWSEWSQCDPCLRQ
MFRSRSIEVFGQFNGKRCTDAVGDRRQCVPTEPCEDAEDDCGNDFQCSTGRCIKMRLRCN
GDNDCGDFSDEDDCESEPRPPCRDRVVEESELARTAGYGINILGMDPLSTPFDNEFYNGL
CNRDRDGNTLTYYRRPWNVASLIYETKGEKNFRTEHYEEQIEAFKSIIQEKTSNFNAAIS
LKFTPTETNKAEQCCEETASSISLHGKGSFRFSYSKNETYQLFLSYSSKKEKMFLHVKGE
IHLGRFVMRNRDVVLTTTFVDDIKALPTTYEKGEYFAFLETYGTHYSSSGSLGGLYELIY
VLDKASMKRKGVELKDIKRCLGYHLDVSLAFSEISVGAEFNKDDCVKRGEGRAVNITSEN
LIDDVVSLIRGGTRKYAFELKEKLLRGTVIDVTDFVNWASSINDAPVLISQKLSPIYNLV
PVKMKNAHLKKQNLERAIEDYINEFSVRKCHTCQNGGTVILMDGKCLCACPFKFEGIACE
ISKQKISEGLPALEFPNEK
Number of residues
559
Molecular Weight
63172.72
Theoretical pI
Not Available
GO Classification
Processes
cell killing / complement activation, alternative pathway / complement activation, classical pathway / protein homooligomerization
Components
blood microparticle / extracellular exosome / extracellular region / extracellular space / membrane attack complex / other organism cell membrane / plasma membrane
General Function
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells (PubMed:26841934, PubMed:9212048, PubMed:9634479). C9 is the pore-forming subunit of the MAC (PubMed:26841934, PubMed:30111885, PubMed:4055801)
Specific Function
Not Available
Pfam Domain Function
Signal Regions
1-21
Transmembrane Regions
314-330 335-354
Cellular Location
Secreted
Gene sequence
>lcl|BSEQ0049817|Complement component C9 (C9)
ATGTCAGCCTGCCGGAGCTTTGCAGTTGCAATCTGCATTTTAGAAATAAGCATCCTCACA
GCACAGTACACGACCAGTTATGACCCAGAGCTAACAGAAAGCAGTGGCTCTGCATCACAC
ATAGACTGCAGAATGAGCCCCTGGAGTGAATGGTCACAATGCGATCCTTGTCTCAGACAA
ATGTTTCGTTCAAGAAGCATTGAGGTCTTTGGACAATTTAATGGGAAAAGATGCACCGAC
GCTGTGGGAGACAGACGACAGTGTGTGCCCACAGAGCCCTGTGAGGATGCTGAGGATGAC
TGCGGAAATGACTTTCAATGCAGTACAGGCAGATGCATAAAGATGCGACTTCGGTGTAAT
GGTGACAATGACTGCGGAGACTTTTCAGATGAGGATGATTGTGAAAGTGAGCCCCGTCCC
CCCTGCAGAGACAGAGTGGTAGAAGAGTCTGAGCTGGCACGAACAGCAGGCTATGGGATC
AACATTTTAGGGATGGATCCCCTAAGCACACCTTTTGACAATGAGTTCTACAATGGACTC
TGTAACCGGGATCGGGATGGAAACACTCTGACATACTACCGAAGACCTTGGAACGTGGCT
TCTTTGATCTATGAAACCAAAGGCGAGAAAAATTTCAGAACCGAACATTACGAAGAACAA
ATTGAAGCATTTAAAAGTATCATCCAAGAGAAGACATCAAATTTTAATGCAGCTATATCT
CTAAAATTTACACCCACTGAAACAAATAAAGCTGAACAATGTTGTGAGGAAACAGCCTCC
TCAATTTCTTTACATGGCAAGGGTAGTTTTCGGTTTTCATATTCCAAAAATGAAACTTAC
CAACTATTTTTGTCATATTCTTCAAAGAAGGAAAAAATGTTTCTGCATGTGAAAGGAGAA
ATTCATCTGGGAAGATTTGTAATGAGAAATCGCGATGTTGTGCTCACAACAACTTTTGTG
GATGATATAAAAGCTTTGCCAACTACCTATGAAAAGGGAGAATATTTTGCCTTTTTGGAA
ACCTATGGAACTCACTACAGTAGCTCTGGGTCTCTAGGAGGACTCTATGAACTAATATAT
GTTTTGGATAAAGCTTCCATGAAGCGGAAAGGTGTTGAACTAAAAGACATAAAGAGATGC
CTTGGGTATCATCTGGATGTATCTCTGGCTTTCTCTGAAATCTCTGTTGGAGCTGAATTT
AATAAAGATGATTGTGTAAAGAGGGGAGAGGGTAGAGCTGTAAACATCACCAGTGAAAAC
CTCATAGATGATGTTGTTTCACTCATAAGAGGTGGAACCAGAAAATATGCATTTGAACTG
AAAGAAAAGCTTCTCCGAGGAACCGTGATTGATGTGACTGACTTTGTCAACTGGGCCTCT
TCCATAAATGATGCTCCTGTTCTCATTAGTCAAAAACTGTCTCCTATATATAATCTGGTT
CCAGTGAAAATGAAAAATGCACACCTAAAGAAACAAAACTTGGAAAGAGCCATTGAAGAC
TATATCAATGAATTTAGTGTAAGAAAATGCCACACATGCCAAAATGGAGGTACAGTGATT
CTAATGGATGGAAAGTGTTTGTGTGCCTGCCCATTCAAATTTGAGGGAATTGCCTGTGAA
ATCAGTAAACAAAAAATTTCTGAAGGATTGCCAGCCCTAGAGTTCCCCAATGAAAAATAG
Chromosome Location
5
Locus
5p13.1
External Identifiers
ResourceLink
UniProtKB IDP02748
UniProtKB Entry NameCO9_HUMAN
GeneCard IDC9
HGNC IDHGNC:1358
PDB ID(s)5FMW, 6DLW, 6H03, 6H04, 7NYC, 7NYD, 8B0G, 8B0H, 8DE6
KEGG IDhsa:735
NCBI Gene ID735
General References
  1. Stanley KK, Kocher HP, Luzio JP, Jackson P, Tschopp J: The sequence and topology of human complement component C9. EMBO J. 1985 Feb;4(2):375-82. [Article]
  2. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  3. DiScipio RG, Gehring MR, Podack ER, Kan CC, Hugli TE, Fey GH: Nucleotide sequence of cDNA and derived amino acid sequence of human complement component C9. Proc Natl Acad Sci U S A. 1984 Dec;81(23):7298-302. [Article]
  4. Marazziti D, Eggertsen G, Fey GH, Stanley KK: Relationships between the gene and protein structure in human complement component C9. Biochemistry. 1988 Aug 23;27(17):6529-34. [Article]
  5. Witzel-Schlomp K, Hobart MJ, Fernie BA, Orren A, Wurzner R, Rittner C, Kaufmann T, Schneider PM: Heterogeneity in the genetic basis of human complement C9 deficiency. Immunogenetics. 1998 Jul;48(2):144-7. [Article]
  6. DiScipio RG, Hugli TE: The architecture of complement component C9 and poly(C9). J Biol Chem. 1985 Nov 25;260(27):14802-9. [Article]
  7. Lengweiler S, Schaller J, Rickli EE: Identification of disulfide bonds in the ninth component (C9) of human complement. FEBS Lett. 1996 Feb 12;380(1-2):8-12. [Article]
  8. Lassiter HA, Walz BM, Wilson JL, Jung E, Calisi CR, Goldsmith LJ, Wilson RA, Morgan BP, Feldhoff RC: The administration of complement component C9 enhances the survival of neonatal rats with Escherichia coli sepsis. Pediatr Res. 1997 Jul;42(1):128-36. [Article]
  9. Hofsteenge J, Blommers M, Hess D, Furmanek A, Miroshnichenko O: The four terminal components of the complement system are C-mannosylated on multiple tryptophan residues. J Biol Chem. 1999 Nov 12;274(46):32786-94. [Article]
  10. Bunkenborg J, Pilch BJ, Podtelejnikov AV, Wisniewski JR: Screening for N-glycosylated proteins by liquid chromatography mass spectrometry. Proteomics. 2004 Feb;4(2):454-65. [Article]
  11. Liu T, Qian WJ, Gritsenko MA, Camp DG 2nd, Monroe ME, Moore RJ, Smith RD: Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry. J Proteome Res. 2005 Nov-Dec;4(6):2070-80. [Article]
  12. Lewandrowski U, Moebius J, Walter U, Sickmann A: Elucidation of N-glycosylation sites on human platelet proteins: a glycoproteomic approach. Mol Cell Proteomics. 2006 Feb;5(2):226-33. Epub 2005 Oct 31. [Article]
  13. Chen R, Jiang X, Sun D, Han G, Wang F, Ye M, Wang L, Zou H: Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J Proteome Res. 2009 Feb;8(2):651-61. doi: 10.1021/pr8008012. [Article]
  14. Jia W, Lu Z, Fu Y, Wang HP, Wang LH, Chi H, Yuan ZF, Zheng ZB, Song LN, Han HH, Liang YM, Wang JL, Cai Y, Zhang YK, Deng YL, Ying WT, He SM, Qian XH: A strategy for precise and large scale identification of core fucosylated glycoproteins. Mol Cell Proteomics. 2009 May;8(5):913-23. doi: 10.1074/mcp.M800504-MCP200. Epub 2009 Jan 12. [Article]
  15. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [Article]
  16. Peitsch MC, Amiguet P, Guy R, Brunner J, Maizel JV Jr, Tschopp J: Localization and molecular modelling of the membrane-inserted domain of the ninth component of human complement and perforin. Mol Immunol. 1990 Jul;27(7):589-602. [Article]
  17. Hadders MA, Bubeck D, Roversi P, Hakobyan S, Forneris F, Morgan BP, Pangburn MK, Llorca O, Lea SM, Gros P: Assembly and regulation of the membrane attack complex based on structures of C5b6 and sC5b9. Cell Rep. 2012 Mar 29;1(3):200-7. doi: 10.1016/j.celrep.2012.02.003. Epub 2012 Feb 23. [Article]
  18. Dudkina NV, Spicer BA, Reboul CF, Conroy PJ, Lukoyanova N, Elmlund H, Law RH, Ekkel SM, Kondos SC, Goode RJ, Ramm G, Whisstock JC, Saibil HR, Dunstone MA: Structure of the poly-C9 component of the complement membrane attack complex. Nat Commun. 2016 Feb 4;7:10588. doi: 10.1038/ncomms10588. [Article]
  19. Seddon JM, Yu Y, Miller EC, Reynolds R, Tan PL, Gowrisankar S, Goldstein JI, Triebwasser M, Anderson HE, Zerbib J, Kavanagh D, Souied E, Katsanis N, Daly MJ, Atkinson JP, Raychaudhuri S: Rare variants in CFI, C3 and C9 are associated with high risk of advanced age-related macular degeneration. Nat Genet. 2013 Nov;45(11):1366-70. doi: 10.1038/ng.2741. Epub 2013 Sep 15. [Article]

Associated Data

Bio-Entities
Bio-EntityType
Complement component C9 (Humans)protein
primary
Drug Relations
DrugDrug groupPharmacological action?TypeActionsDetails
Copperapproved, investigationalunknowntargetDetails