Complement C5

Details

Name
Complement C5
Synonyms
  • C3 and PZP-like alpha-2-macroglobulin domain-containing protein 4
  • CPAMD4
Gene Name
C5
Organism
Humans
Amino acid sequence
>lcl|BSEQ0019020|Complement C5
MGLLGILCFLIFLGKTWGQEQTYVISAPKIFRVGASENIVIQVYGYTEAFDATISIKSYP
DKKFSYSSGHVHLSSENKFQNSAILTIQPKQLPGGQNPVSYVYLEVVSKHFSKSKRMPIT
YDNGFLFIHTDKPVYTPDQSVKVRVYSLNDDLKPAKRETVLTFIDPEGSEVDMVEEIDHI
GIISFPDFKIPSNPRYGMWTIKAKYKEDFSTTGTAYFEVKEYVLPHFSVSIEPEYNFIGY
KNFKNFEITIKARYFYNKVVTEADVYITFGIREDLKDDQKEMMQTAMQNTMLINGIAQVT
FDSETAVKELSYYSLEDLNNKYLYIAVTVIESTGGFSEEAEIPGIKYVLSPYKLNLVATP
LFLKPGIPYPIKVQVKDSLDQLVGGVPVTLNAQTIDVNQETSDLDPSKSVTRVDDGVASF
VLNLPSGVTVLEFNVKTDAPDLPEENQAREGYRAIAYSSLSQSYLYIDWTDNHKALLVGE
HLNIIVTPKSPYIDKITHYNYLILSKGKIIHFGTREKFSDASYQSINIPVTQNMVPSSRL
LVYYIVTGEQTAELVSDSVWLNIEEKCGNQLQVHLSPDADAYSPGQTVSLNMATGMDSWV
ALAAVDSAVYGVQRGAKKPLERVFQFLEKSDLGCGAGGGLNNANVFHLAGLTFLTNANAD
DSQENDEPCKEILRPRRTLQKKIEEIAAKYKHSVVKKCCYDGACVNNDETCEQRAARISL
GPRCIKAFTECCVVASQLRANISHKDMQLGRLHMKTLLPVSKPEIRSYFPESWLWEVHLV
PRRKQLQFALPDSLTTWEIQGVGISNTGICVADTVKAKVFKDVFLEMNIPYSVVRGEQIQ
LKGTVYNYRTSGMQFCVKMSAVEGICTSESPVIDHQGTKSSKCVRQKVEGSSSHLVTFTV
LPLEIGLHNINFSLETWFGKEILVKTLRVVPEGVKRESYSGVTLDPRGIYGTISRRKEFP
YRIPLDLVPKTEIKRILSVKGLLVGEILSAVLSQEGINILTHLPKGSAEAELMSVVPVFY
VFHYLETGNHWNIFHSDPLIEKQKLKKKLKEGMLSIMSYRNADYSYSVWKGGSASTWLTA
FALRVLGQVNKYVEQNQNSICNSLLWLVENYQLDNGSFKENSQYQPIKLQGTLPVEAREN
SLYLTAFTVIGIRKAFDICPLVKIDTALIKADNFLLENTLPAQSTFTLAISAYALSLGDK
THPQFRSIVSALKREALVKGNPPIYRFWKDNLQHKDSSVPNTGTARMVETTAYALLTSLN
LKDINYVNPVIKWLSEEQRYGGGFYSTQDTINAIEGLTEYSLLVKQLRLSMDIDVSYKHK
GALHNYKMTDKNFLGRPVEVLLNDDLIVSTGFGSGLATVHVTTVVHKTSTSEEVCSFYLK
IDTQDIEASHYRGYGNSDYKRIVACASYKPSREESSSGSSHAVMDISLPTGISANEEDLK
ALVEGVDQLFTDYQIKDGHVILQLNSIPSSDFLCVRFRIFELFEVGFLSPATFTVYEYHR
PDKQCTMFYSTSNIKIQKVCEGAACKCVEADCGQMQEELDLTISAETRKQTACKPEIAYA
YKVSITSITVENVFVKYKATLLDIYKTGEAVAEKDSEITFIKKVTCTNAELVKGRQYLIM
GKEALQIKYNFSFRYIYPLDSLTWIEYWPRDTTCSSCQAFLANLDEFAEDIFLNGC
Number of residues
1676
Molecular Weight
188303.705
Theoretical pI
6.49
GO Classification
Functions
chemokine activity / endopeptidase inhibitor activity / receptor binding
Processes
activation of MAPK activity / cell chemotaxis / cell surface receptor signaling pathway / chemotaxis / complement activation / complement activation, alternative pathway / complement activation, classical pathway / cytolysis / G-protein coupled receptor signaling pathway / in utero embryonic development / inflammatory response / innate immune response / mitophagy in response to mitochondrial depolarization / negative regulation of macrophage chemotaxis / positive regulation of angiogenesis / positive regulation of chemokine secretion / positive regulation of vascular endothelial growth factor production / regulation of complement activation / response to stress
Components
extracellular exosome / extracellular region / extracellular space / membrane attack complex
General Function
Receptor binding
Specific Function
Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. C5b has a transient binding site for C6. The C5b-C6 complex is the foundation upon which the lytic complex is assembled.Derived from proteolytic degradation of complement C5, C5 anaphylatoxin is a mediator of local inflammatory process. Binding to the receptor C5AR1 induces a variety of responses including intracellular calcium release, contraction of smooth muscle, increased vascular permeability, and histamine release from mast cells and basophilic leukocytes (PubMed:8182049). C5a is also a potent chemokine which stimulates the locomotion of polymorphonuclear leukocytes and directs their migration toward sites of inflammation.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Secreted
Gene sequence
>lcl|BSEQ0019021|Complement C5 (C5)
ATGGGCCTTTTGGGAATACTTTGTTTTTTAATCTTCCTGGGGAAAACCTGGGGACAGGAG
CAAACATATGTCATTTCAGCACCAAAAATATTCCGTGTTGGAGCATCTGAAAATATTGTG
ATTCAAGTTTATGGATACACTGAAGCATTTGATGCAACAATCTCTATTAAAAGTTATCCT
GATAAAAAATTTAGTTACTCCTCAGGCCATGTTCATTTATCCTCAGAGAATAAATTCCAA
AACTCTGCAATCTTAACAATACAACCAAAACAATTGCCTGGAGGACAAAACCCAGTTTCT
TATGTGTATTTGGAAGTTGTATCAAAGCATTTTTCAAAATCAAAAAGAATGCCAATAACC
TATGACAATGGATTTCTCTTCATTCATACAGACAAACCTGTTTATACTCCAGACCAGTCA
GTAAAAGTTAGAGTTTATTCGTTGAATGACGACTTGAAGCCAGCCAAAAGAGAAACTGTC
TTAACTTTCATAGATCCTGAAGGATCAGAAGTTGACATGGTAGAAGAAATTGATCATATT
GGAATTATCTCTTTTCCTGACTTCAAGATTCCGTCTAATCCTAGATATGGTATGTGGACG
ATCAAGGCTAAATATAAAGAGGACTTTTCAACAACTGGAACCGCATATTTTGAAGTTAAA
GAATATGTCTTGCCACATTTTTCTGTCTCAATCGAGCCAGAATATAATTTCATTGGTTAC
AAGAACTTTAAGAATTTTGAAATTACTATAAAAGCAAGATATTTTTATAATAAAGTAGTC
ACTGAGGCTGACGTTTATATCACATTTGGAATAAGAGAAGACTTAAAAGATGATCAAAAA
GAAATGATGCAAACAGCAATGCAAAACACAATGTTGATAAATGGAATTGCTCAAGTCACA
TTTGATTCTGAAACAGCAGTCAAAGAACTGTCATACTACAGTTTAGAAGATTTAAACAAC
AAGTACCTTTATATTGCTGTAACAGTCATAGAGTCTACAGGTGGATTTTCTGAAGAGGCA
GAAATACCTGGCATCAAATATGTCCTCTCTCCCTACAAACTGAATTTGGTTGCTACTCCT
CTTTTCCTGAAGCCTGGGATTCCATATCCCATCAAGGTGCAGGTTAAAGATTCGCTTGAC
CAGTTGGTAGGAGGAGTCCCAGTAACACTGAATGCACAAACAATTGATGTAAACCAAGAG
ACATCTGACTTGGATCCAAGCAAAAGTGTAACACGTGTTGATGATGGAGTAGCTTCCTTT
GTGCTTAATCTCCCATCTGGAGTGACGGTGCTGGAGTTTAATGTCAAAACTGATGCTCCA
GATCTTCCAGAAGAAAATCAGGCCAGGGAAGGTTACCGAGCAATAGCATACTCATCTCTC
AGCCAAAGTTACCTTTATATTGATTGGACTGATAACCATAAGGCTTTGCTAGTGGGAGAA
CATCTGAATATTATTGTTACCCCCAAAAGCCCATATATTGACAAAATAACTCACTATAAT
TACTTGATTTTATCCAAGGGCAAAATTATCCACTTTGGCACGAGGGAGAAATTTTCAGAT
GCATCTTATCAAAGTATAAACATTCCAGTAACACAGAACATGGTTCCTTCATCCCGACTT
CTGGTCTATTACATCGTCACAGGAGAACAGACAGCAGAATTAGTGTCTGATTCAGTCTGG
TTAAATATTGAAGAAAAATGTGGCAACCAGCTCCAGGTTCATCTGTCTCCTGATGCAGAT
GCATATTCTCCAGGCCAAACTGTGTCTCTTAATATGGCAACTGGAATGGATTCCTGGGTG
GCATTAGCAGCAGTGGACAGTGCTGTGTATGGAGTCCAAAGAGGAGCCAAAAAGCCCTTG
GAAAGAGTATTTCAATTCTTAGAGAAGAGTGATCTGGGCTGTGGGGCAGGTGGTGGCCTC
AACAATGCCAATGTGTTCCACCTAGCTGGACTTACCTTCCTCACTAATGCAAATGCAGAT
GACTCCCAAGAAAATGATGAACCTTGTAAAGAAATTCTCAGGCCAAGAAGAACGCTGCAA
AAGAAGATAGAAGAAATAGCTGCTAAATATAAACATTCAGTAGTGAAGAAATGTTGTTAC
GATGGAGCCTGCGTTAATAATGATGAAACCTGTGAGCAGCGAGCTGCACGGATTAGTTTA
GGGCCAAGATGCATCAAAGCTTTCACTGAATGTTGTGTCGTCGCAAGCCAGCTCCGTGCT
AATATCTCTCATAAAGACATGCAATTGGGAAGGCTACACATGAAGACCCTGTTACCAGTA
AGCAAGCCAGAAATTCGGAGTTATTTTCCAGAAAGCTGGTTGTGGGAAGTTCATCTTGTT
CCCAGAAGAAAACAGTTGCAGTTTGCCCTACCTGATTCTCTAACCACCTGGGAAATTCAA
GGCGTTGGCATTTCAAACACTGGTATATGTGTTGCTGATACTGTCAAGGCAAAGGTGTTC
AAAGATGTCTTCCTGGAAATGAATATACCATATTCTGTTGTACGAGGAGAACAGATCCAA
TTGAAAGGAACTGTTTACAACTATAGGACTTCTGGGATGCAGTTCTGTGTTAAAATGTCT
GCTGTGGAGGGAATCTGCACTTCGGAAAGCCCAGTCATTGATCATCAGGGCACAAAGTCC
TCCAAATGTGTGCGCCAGAAAGTAGAGGGCTCCTCCAGTCACTTGGTGACATTCACTGTG
CTTCCTCTGGAAATTGGCCTTCACAACATCAATTTTTCACTGGAGACTTGGTTTGGAAAA
GAAATCTTAGTAAAAACATTACGAGTGGTGCCAGAAGGTGTCAAAAGGGAAAGCTATTCT
GGTGTTACTTTGGATCCTAGGGGTATTTATGGTACCATTAGCAGACGAAAGGAGTTCCCA
TACAGGATACCCTTAGATTTGGTCCCCAAAACAGAAATCAAAAGGATTTTGAGTGTAAAA
GGACTGCTTGTAGGTGAGATCTTGTCTGCAGTTCTAAGTCAGGAAGGCATCAATATCCTA
ACCCACCTCCCCAAAGGGAGTGCAGAGGCGGAGCTGATGAGCGTTGTCCCAGTATTCTAT
GTTTTTCACTACCTGGAAACAGGAAATCATTGGAACATTTTTCATTCTGACCCATTAATT
GAAAAGCAGAAACTGAAGAAAAAATTAAAAGAAGGGATGTTGAGCATTATGTCCTACAGA
AATGCTGACTACTCTTACAGTGTGTGGAAGGGTGGAAGTGCTAGCACTTGGTTAACAGCT
TTTGCTTTAAGAGTACTTGGACAAGTAAATAAATACGTAGAGCAGAACCAAAATTCAATT
TGTAATTCTTTATTGTGGCTAGTTGAGAATTATCAATTAGATAATGGATCTTTCAAGGAA
AATTCACAGTATCAACCAATAAAATTACAGGGTACCTTGCCTGTTGAAGCCCGAGAGAAC
AGCTTATATCTTACAGCCTTTACTGTGATTGGAATTAGAAAGGCTTTCGATATATGCCCC
CTGGTGAAAATCGACACAGCTCTAATTAAAGCTGACAACTTTCTGCTTGAAAATACACTG
CCAGCCCAGAGCACCTTTACATTGGCCATTTCTGCGTATGCTCTTTCCCTGGGAGATAAA
ACTCACCCACAGTTTCGTTCAATTGTTTCAGCTTTGAAGAGAGAAGCTTTGGTTAAAGGT
AATCCACCCATTTATCGTTTTTGGAAAGACAATCTTCAGCATAAAGACAGCTCTGTACCT
AACACTGGTACGGCACGTATGGTAGAAACAACTGCCTATGCTTTACTCACCAGTCTGAAC
TTGAAAGATATAAATTATGTTAACCCAGTCATCAAATGGCTATCAGAAGAGCAGAGGTAT
GGAGGTGGCTTTTATTCAACCCAGGACACAATCAATGCCATTGAGGGCCTGACGGAATAT
TCACTCCTGGTTAAACAACTCCGCTTGAGTATGGACATCGATGTTTCTTACAAGCATAAA
GGTGCCTTACATAATTATAAAATGACAGACAAGAATTTCCTTGGGAGGCCAGTAGAGGTG
CTTCTCAATGATGACCTCATTGTCAGTACAGGATTTGGCAGTGGCTTGGCTACAGTACAT
GTAACAACTGTAGTTCACAAAACCAGTACCTCTGAGGAAGTTTGCAGCTTTTATTTGAAA
ATCGATACTCAGGATATTGAAGCATCCCACTACAGAGGCTACGGAAACTCTGATTACAAA
CGCATAGTAGCATGTGCCAGCTACAAGCCCAGCAGGGAAGAATCATCATCTGGATCCTCT
CATGCGGTGATGGACATCTCCTTGCCTACTGGAATCAGTGCAAATGAAGAAGACTTAAAA
GCCCTTGTGGAAGGGGTGGATCAACTATTCACTGATTACCAAATCAAAGATGGACATGTT
ATTCTGCAACTGAATTCGATTCCCTCCAGTGATTTCCTTTGTGTACGATTCCGGATATTT
GAACTCTTTGAAGTTGGGTTTCTCAGTCCTGCCACTTTCACAGTGTACGAATACCACAGA
CCAGATAAACAGTGTACCATGTTTTATAGCACTTCCAATATCAAAATTCAGAAAGTCTGT
GAAGGAGCCGCGTGCAAGTGTGTAGAAGCTGATTGTGGGCAAATGCAGGAAGAATTGGAT
CTGACAATCTCTGCAGAGACAAGAAAACAAACAGCATGTAAACCAGAGATTGCATATGCT
TATAAAGTTAGCATCACATCCATCACTGTAGAAAATGTTTTTGTCAAGTACAAGGCAACC
CTTCTGGATATCTACAAAACTGGGGAAGCTGTTGCTGAGAAAGACTCTGAGATTACCTTC
ATTAAAAAGGTAACCTGTACTAACGCTGAGCTGGTAAAAGGAAGACAGTACTTAATTATG
GGTAAAGAAGCCCTCCAGATAAAATACAATTTCAGTTTCAGGTACATCTACCCTTTAGAT
TCCTTGACCTGGATTGAATACTGGCCTAGAGACACAACATGTTCATCGTGTCAAGCATTT
TTAGCTAATTTAGATGAATTTGCCGAAGATATCTTTTTAAATGGATGCTAA
Chromosome Location
9
Locus
9q33-q34
External Identifiers
ResourceLink
UniProtKB IDP01031
UniProtKB Entry NameCO5_HUMAN
GenBank Protein ID179983
GenBank Gene IDM57729
GenAtlas IDC5
HGNC IDHGNC:1331
General References
  1. Haviland DL, Haviland JC, Fleischer DT, Hunt A, Wetsel RA: Complete cDNA sequence of human complement pro-C5. Evidence of truncated transcripts derived from a single copy gene. J Immunol. 1991 Jan 1;146(1):362-8. [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. Wetsel RA, Lemons RS, Le Beau MM, Barnum SR, Noack D, Tack BF: Molecular analysis of human complement component C5: localization of the structural gene to chromosome 9. Biochemistry. 1988 Mar 8;27(5):1474-82. [Article]
  4. Lundwall AB, Wetsel RA, Kristensen T, Whitehead AS, Woods DE, Ogden RC, Colten HR, Tack BF: Isolation and sequence analysis of a cDNA clone encoding the fifth complement component. J Biol Chem. 1985 Feb 25;260(4):2108-12. [Article]
  5. Fernandez HN, Hugli TE: Primary structural analysis of the polypeptide portion of human C5a anaphylatoxin. Polypeptide sequence determination and assignment of the oligosaccharide attachment site in C5a. J Biol Chem. 1978 Oct 10;253(19):6955-64. [Article]
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  8. Chen Z, Zhang X, Gonnella NC, Pellas TC, Boyar WC, Ni F: Residues 21-30 within the extracellular N-terminal region of the C5a receptor represent a binding domain for the C5a anaphylatoxin. J Biol Chem. 1998 Apr 24;273(17):10411-9. [Article]
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  12. Zuiderweg ER, Fesik SW: Heteronuclear three-dimensional NMR spectroscopy of the inflammatory protein C5a. Biochemistry. 1989 Mar 21;28(6):2387-91. [Article]
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  14. Zhang X, Boyar W, Toth MJ, Wennogle L, Gonnella NC: Structural definition of the C5a C terminus by two-dimensional nuclear magnetic resonance spectroscopy. Proteins. 1997 Jun;28(2):261-7. [Article]
  15. Wang X, Fleischer DT, Whitehead WT, Haviland DL, Rosenfeld SI, Leddy JP, Snyderman R, Wetsel RA: Inherited human complement C5 deficiency. Nonsense mutations in exons 1 (Gln1 to Stop) and 36 (Arg1458 to Stop) and compound heterozygosity in three African-American families. J Immunol. 1995 May 15;154(10):5464-71. [Article]
  16. Pfarr N, Prawitt D, Kirschfink M, Schroff C, Knuf M, Habermehl P, Mannhardt W, Zepp F, Fairbrother WG, Loos M, Burge CB, Pohlenz J: Linking C5 deficiency to an exonic splicing enhancer mutation. J Immunol. 2005 Apr 1;174(7):4172-7. [Article]
  17. Delgado-Cervino E, Fontan G, Lopez-Trascasa M: C5 complement deficiency in a Spanish family. Molecular characterization of the double mutation responsible for the defect. Mol Immunol. 2005 Jan;42(1):105-11. [Article]
  18. Hillebrandt S, Wasmuth HE, Weiskirchen R, Hellerbrand C, Keppeler H, Werth A, Schirin-Sokhan R, Wilkens G, Geier A, Lorenzen J, Kohl J, Gressner AM, Matern S, Lammert F: Complement factor 5 is a quantitative trait gene that modifies liver fibrogenesis in mice and humans. Nat Genet. 2005 Aug;37(8):835-43. Epub 2005 Jul 3. [Article]
  19. Bramham J, Thai CT, Soares DC, Uhrin D, Ogata RT, Barlow PN: Functional insights from the structure of the multifunctional C345C domain of C5 of complement. J Biol Chem. 2005 Mar 18;280(11):10636-45. Epub 2004 Dec 14. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB01257Eculizumabapproved, investigationalyesantibodyDetails
DB00028Human immunoglobulin Gapproved, investigationalyesbinderDetails
DB09130Copperapproved, investigationalunknownDetails
DB01593Zincapproved, investigationalunknownDetails
DB14487Zinc acetateapproved, investigationalunknownDetails
DB14533Zinc chlorideapproved, investigationalunknownligandDetails
DB11580Ravulizumabapproved, investigationalyesinhibitorDetails
DB14548Zinc sulfate, unspecified formapproved, experimentalunknownligandDetails
DB16416VilobelimabinvestigationalunknowninhibitorDetails
DB15636ZilucoplanapprovedyesinhibitorDetails
DB15165Avacincaptad pegolapproved, investigationalyesinhibitorDetails
DB15218Pozelimabapproved, investigationalyesantibodyDetails