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Protein

Complement component C7

Gene

C7

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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. C7 serves as a membrane anchor.

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Complement alternate pathway, Complement pathway, Cytolysis, Immunity, Innate immunity

Enzyme and pathway databases

BioCyciZFISH:ENSG00000112936-MONOMER.
ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
Complement component C7
Gene namesi
Name:C7
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:1346. C7.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • extracellular region Source: Reactome
  • membrane attack complex Source: ProtInc
Complete GO annotation...

Keywords - Cellular componenti

Membrane attack complex, Secreted

Pathology & Biotechi

Involvement in diseasei

Complement component 7 deficiency (C7D)3 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
See also OMIM:610102
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_012643220R → Q in C7D. 1 PublicationCorresponds to variant rs369349760dbSNPEnsembl.1
Natural variantiVAR_012644379G → R in C7D. 1 PublicationCorresponds to variant rs121964921dbSNPEnsembl.1
Natural variantiVAR_012645521R → S in C7D. 1 PublicationCorresponds to variant rs121964920dbSNPEnsembl.1
Natural variantiVAR_012646682E → Q in C7D. 1 PublicationCorresponds to variant rs541873000dbSNPEnsembl.1
Natural variantiVAR_012647687R → H in C7D. 1 PublicationCorresponds to variant rs113187203dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi730.
MalaCardsiC7.
MIMi610102. phenotype.
OpenTargetsiENSG00000112936.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25941.

Polymorphism and mutation databases

BioMutaiC7.
DMDMi61252057.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 22Add BLAST22
ChainiPRO_000002358323 – 843Complement component C7Add BLAST821

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi36C-linked (Man)1 Publication1
Disulfide bondi85 ↔ 96By similarity
Disulfide bondi91 ↔ 109By similarity
Disulfide bondi103 ↔ 119By similarity
Disulfide bondi128 ↔ 165By similarity
Glycosylationi202N-linked (GlcNAc...)2 Publications1
Disulfide bondi337 ↔ 3531 Publication
Glycosylationi503C-linked (Man); partial1 Publication1
Glycosylationi506C-linked (Man); partial1 Publication1
Glycosylationi509C-linked (Man); partial1 Publication1
Disulfide bondi571 ↔ 613By similarity
Disulfide bondi599 ↔ 626By similarity
Disulfide bondi631 ↔ 673By similarity
Disulfide bondi659 ↔ 688By similarity
Glycosylationi696O-linked (GalNAc...)1 Publication1
Disulfide bondi702 ↔ 7131 Publication
Disulfide bondi715 ↔ 7501 Publication
Disulfide bondi721 ↔ 7431 Publication
Disulfide bondi728 ↔ 7631 Publication
Glycosylationi754N-linked (GlcNAc...) (complex)2 Publications1
Disulfide bondi773 ↔ 7821 Publication
Disulfide bondi776 ↔ 7891 Publication
Disulfide bondi791 ↔ 8251 Publication
Disulfide bondi797 ↔ 8181 Publication
Disulfide bondi805 ↔ 8381 Publication

Post-translational modificationi

C7 has 28 disulfide bridges.
C-, N- and O-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans.5 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP10643.
PeptideAtlasiP10643.
PRIDEiP10643.

PTM databases

iPTMnetiP10643.
PhosphoSitePlusiP10643.
UniCarbKBiP10643.

Expressioni

Gene expression databases

BgeeiENSG00000112936.
CleanExiHS_C7.
GenevisibleiP10643. HS.

Organism-specific databases

HPAiHPA001465.

Interactioni

Subunit structurei

Monomer or dimer; as a C5b-7 complex it can also form multimeric rosettes. MAC assembly is initiated by proteolytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and multiple copies of the pore-forming subunit C9.

Protein-protein interaction databases

BioGridi107191. 4 interactors.
IntActiP10643. 3 interactors.
MINTiMINT-7032139.
STRINGi9606.ENSP00000322061.

Structurei

Secondary structure

1843
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi706 – 709Combined sources4
Beta strandi712 – 715Combined sources4
Helixi718 – 720Combined sources3
Beta strandi726 – 731Combined sources6
Turni732 – 734Combined sources3
Beta strandi737 – 741Combined sources5
Helixi742 – 750Combined sources9
Beta strandi755 – 757Combined sources3
Beta strandi766 – 769Combined sources4
Beta strandi778 – 782Combined sources5
Beta strandi784 – 791Combined sources8
Helixi794 – 796Combined sources3
Beta strandi803 – 808Combined sources6
Beta strandi811 – 816Combined sources6
Helixi817 – 826Combined sources10
Beta strandi831 – 836Combined sources6

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2WCYNMR-A693-843[»]
ProteinModelPortaliP10643.
SMRiP10643.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP10643.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini27 – 80TSP type-1 1PROSITE-ProRule annotationAdd BLAST54
Domaini83 – 121LDL-receptor class APROSITE-ProRule annotationAdd BLAST39
Domaini124 – 456MACPFPROSITE-ProRule annotationAdd BLAST333
Domaini457 – 487EGF-likeAdd BLAST31
Domaini500 – 549TSP type-1 2PROSITE-ProRule annotationAdd BLAST50
Domaini569 – 628Sushi 1PROSITE-ProRule annotationAdd BLAST60
Domaini629 – 690Sushi 2PROSITE-ProRule annotationAdd BLAST62

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni545 – 615CCP 1Add BLAST71
Regioni616 – 693CCP 2Add BLAST78
Regioni695 – 770Factor I module (FIM) 1Add BLAST76
Regioni771 – 843Factor I module (FIM) 2Add BLAST73

Sequence similaritiesi

Belongs to the complement C6/C7/C8/C9 family.Curated
Contains 1 EGF-like domain.Curated
Contains 1 LDL-receptor class A domain.PROSITE-ProRule annotation
Contains 1 MACPF domain.PROSITE-ProRule annotation
Contains 2 Sushi (CCP/SCR) domains.PROSITE-ProRule annotation
Contains 2 TSP type-1 domains.PROSITE-ProRule annotation

Keywords - Domaini

EGF-like domain, Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiENOG410IDXP. Eukaryota.
ENOG410YJ70. LUCA.
GeneTreeiENSGT00550000074478.
HOGENOMiHOG000111868.
HOVERGENiHBG005367.
InParanoidiP10643.
KOiK03996.
OMAiNYEFYNS.
OrthoDBiEOG091G07LK.
PhylomeDBiP10643.
TreeFamiTF330498.

Family and domain databases

CDDicd00033. CCP. 2 hits.
Gene3Di4.10.400.10. 1 hit.
InterProiIPR003884. FacI_MAC.
IPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000436. Sushi_SCR_CCP_dom.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00084. Sushi. 2 hits.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00032. CCP. 2 hits.
SM00057. FIMAC. 2 hits.
SM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 2 hits.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01186. EGF_2. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50923. SUSHI. 2 hits.
PS50092. TSP1. 2 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P10643-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKVISLFILV GFIGEFQSFS SASSPVNCQW DFYAPWSECN GCTKTQTRRR
60 70 80 90 100
SVAVYGQYGG QPCVGNAFET QSCEPTRGCP TEEGCGERFR CFSGQCISKS
110 120 130 140 150
LVCNGDSDCD EDSADEDRCE DSERRPSCDI DKPPPNIELT GNGYNELTGQ
160 170 180 190 200
FRNRVINTKS FGGQCRKVFS GDGKDFYRLS GNVLSYTFQV KINNDFNYEF
210 220 230 240 250
YNSTWSYVKH TSTEHTSSSR KRSFFRSSSS SSRSYTSHTN EIHKGKSYQL
260 270 280 290 300
LVVENTVEVA QFINNNPEFL QLAEPFWKEL SHLPSLYDYS AYRRLIDQYG
310 320 330 340 350
THYLQSGSLG GEYRVLFYVD SEKLKQNDFN SVEEKKCKSS GWHFVVKFSS
360 370 380 390 400
HGCKELENAL KAASGTQNNV LRGEPFIRGG GAGFISGLSY LELDNPAGNK
410 420 430 440 450
RRYSAWAESV TNLPQVIKQK LTPLYELVKE VPCASVKKLY LKWALEEYLD
460 470 480 490 500
EFDPCHCRPC QNGGLATVEG THCLCHCKPY TFGAACEQGV LVGNQAGGVD
510 520 530 540 550
GGWSCWSSWS PCVQGKKTRS RECNNPPPSG GGRSCVGETT ESTQCEDEEL
560 570 580 590 600
EHLRLLEPHC FPLSLVPTEF CPSPPALKDG FVQDEGTMFP VGKNVVYTCN
610 620 630 640 650
EGYSLIGNPV ARCGEDLRWL VGEMHCQKIA CVLPVLMDGI QSHPQKPFYT
660 670 680 690 700
VGEKVTVSCS GGMSLEGPSA FLCGSSLKWS PEMKNARCVQ KENPLTQAVP
710 720 730 740 750
KCQRWEKLQN SRCVCKMPYE CGPSLDVCAQ DERSKRILPL TVCKMHVLHC
760 770 780 790 800
QGRNYTLTGR DSCTLPASAE KACGACPLWG KCDAESSKCV CREASECEEE
810 820 830 840
GFSICVEVNG KEQTMSECEA GALRCRGQSI SVTSIRPCAA ETQ
Length:843
Mass (Da):93,518
Last modified:March 15, 2005 - v2
Checksum:iDBD5D7C92DF71FA5
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti152R → V in CAA60121 (PubMed:7730625).Curated1
Sequence conflicti821 – 822GA → AL (PubMed:7730625).Curated2

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_050480128C → R.Corresponds to variant rs2271708dbSNPEnsembl.1
Natural variantiVAR_012643220R → Q in C7D. 1 PublicationCorresponds to variant rs369349760dbSNPEnsembl.1
Natural variantiVAR_012644379G → R in C7D. 1 PublicationCorresponds to variant rs121964921dbSNPEnsembl.1
Natural variantiVAR_033798389S → T Polymorphism; confirmed at protein level. 2 PublicationsCorresponds to variant rs1063499dbSNPEnsembl.1
Natural variantiVAR_022023420K → Q.Corresponds to variant rs3792646dbSNPEnsembl.1
Natural variantiVAR_012645521R → S in C7D. 1 PublicationCorresponds to variant rs121964920dbSNPEnsembl.1
Natural variantiVAR_033799587T → P Polymorphism; confirmed at protein level. 2 PublicationsCorresponds to variant rs13157656dbSNPEnsembl.1
Natural variantiVAR_012646682E → Q in C7D. 1 PublicationCorresponds to variant rs541873000dbSNPEnsembl.1
Natural variantiVAR_012647687R → H in C7D. 1 PublicationCorresponds to variant rs113187203dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03507 mRNA. Translation: AAA51861.1.
BC063851 mRNA. Translation: AAH63851.1.
X86328
, X86329, X86330, X86331, X86332, X86333, X86334, X86335, X86336, X86337, X86338, X86339, X86340, X86341, X86342, X86343, X86344 Genomic DNA. Translation: CAA60121.1.
CCDSiCCDS47201.1.
PIRiA27340.
RefSeqiNP_000578.2. NM_000587.2.
UniGeneiHs.78065.

Genome annotation databases

EnsembliENST00000313164; ENSP00000322061; ENSG00000112936.
GeneIDi730.
KEGGihsa:730.
UCSCiuc003jmh.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

C7base

C7 mutation db

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03507 mRNA. Translation: AAA51861.1.
BC063851 mRNA. Translation: AAH63851.1.
X86328
, X86329, X86330, X86331, X86332, X86333, X86334, X86335, X86336, X86337, X86338, X86339, X86340, X86341, X86342, X86343, X86344 Genomic DNA. Translation: CAA60121.1.
CCDSiCCDS47201.1.
PIRiA27340.
RefSeqiNP_000578.2. NM_000587.2.
UniGeneiHs.78065.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2WCYNMR-A693-843[»]
ProteinModelPortaliP10643.
SMRiP10643.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107191. 4 interactors.
IntActiP10643. 3 interactors.
MINTiMINT-7032139.
STRINGi9606.ENSP00000322061.

PTM databases

iPTMnetiP10643.
PhosphoSitePlusiP10643.
UniCarbKBiP10643.

Polymorphism and mutation databases

BioMutaiC7.
DMDMi61252057.

Proteomic databases

PaxDbiP10643.
PeptideAtlasiP10643.
PRIDEiP10643.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000313164; ENSP00000322061; ENSG00000112936.
GeneIDi730.
KEGGihsa:730.
UCSCiuc003jmh.5. human.

Organism-specific databases

CTDi730.
DisGeNETi730.
GeneCardsiC7.
HGNCiHGNC:1346. C7.
HPAiHPA001465.
MalaCardsiC7.
MIMi217070. gene.
610102. phenotype.
neXtProtiNX_P10643.
OpenTargetsiENSG00000112936.
Orphaneti169150. Immunodeficiency due to a late component of complements deficiency.
PharmGKBiPA25941.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IDXP. Eukaryota.
ENOG410YJ70. LUCA.
GeneTreeiENSGT00550000074478.
HOGENOMiHOG000111868.
HOVERGENiHBG005367.
InParanoidiP10643.
KOiK03996.
OMAiNYEFYNS.
OrthoDBiEOG091G07LK.
PhylomeDBiP10643.
TreeFamiTF330498.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000112936-MONOMER.
ReactomeiR-HSA-166665. Terminal pathway of complement.
R-HSA-977606. Regulation of Complement cascade.

Miscellaneous databases

ChiTaRSiC7. human.
EvolutionaryTraceiP10643.
GenomeRNAii730.
PROiP10643.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000112936.
CleanExiHS_C7.
GenevisibleiP10643. HS.

Family and domain databases

CDDicd00033. CCP. 2 hits.
Gene3Di4.10.400.10. 1 hit.
InterProiIPR003884. FacI_MAC.
IPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001862. MAC_perforin.
IPR020864. MACPF.
IPR020863. MACPF_CS.
IPR000436. Sushi_SCR_CCP_dom.
IPR000884. TSP1_rpt.
[Graphical view]
PfamiPF00057. Ldl_recept_a. 1 hit.
PF01823. MACPF. 1 hit.
PF00084. Sushi. 2 hits.
PF00090. TSP_1. 2 hits.
[Graphical view]
PRINTSiPR00764. COMPLEMENTC9.
SMARTiSM00032. CCP. 2 hits.
SM00057. FIMAC. 2 hits.
SM00192. LDLa. 1 hit.
SM00457. MACPF. 1 hit.
SM00209. TSP1. 2 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 2 hits.
SSF82895. SSF82895. 2 hits.
PROSITEiPS00022. EGF_1. 1 hit.
PS01186. EGF_2. 1 hit.
PS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS00279. MACPF_1. 1 hit.
PS51412. MACPF_2. 1 hit.
PS50923. SUSHI. 2 hits.
PS50092. TSP1. 2 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCO7_HUMAN
AccessioniPrimary (citable) accession number: P10643
Secondary accession number(s): Q6P3T5, Q92489
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: March 15, 2005
Last modified: November 30, 2016
This is version 178 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.