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Protein

Complement C2

Gene

C2

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

Functioni

Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor C4b to generate the C3 or C5 convertase.

Catalytic activityi

Selective cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to form C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to form C5a and C5b.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi262Divalent metal cation1
Metal bindingi264Divalent metal cation1
Metal bindingi337Divalent metal cation1
Active sitei507Charge relay system1 Publication1
Active sitei561Charge relay system1 Publication1
Active sitei679Charge relay system1 Publication1

GO - Molecular functioni

GO - Biological processi

  • complement activation Source: BHF-UCL
  • complement activation, classical pathway Source: ProtInc
  • innate immune response Source: UniProtKB-KW
  • positive regulation of apoptotic cell clearance Source: BHF-UCL
  • regulation of complement activation Source: Reactome
  • response to nutrient Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Serine protease

Keywords - Biological processi

Complement pathway, Immunity, Innate immunity

Keywords - Ligandi

Metal-binding

Enzyme and pathway databases

BioCyciZFISH:HS09368-MONOMER.
ReactomeiR-HSA-166663. Initial triggering of complement.
R-HSA-174577. Activation of C3 and C5.
R-HSA-977606. Regulation of Complement cascade.
SABIO-RKP06681.

Protein family/group databases

MEROPSiS01.194.

Names & Taxonomyi

Protein namesi
Recommended name:
Complement C2 (EC:3.4.21.43)
Alternative name(s):
C3/C5 convertase
Cleaved into the following 2 chains:
Gene namesi
Name:C2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:1248. C2.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • extracellular region Source: Reactome
  • extracellular space Source: ProtInc
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Complement component 2 deficiency (C2D)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent invasive infections.
See also OMIM:217000
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_008544131C → Y in C2D. 1 PublicationCorresponds to variant rs760744400dbSNPEnsembl.1
Natural variantiVAR_008545209S → F in C2D. 1 PublicationCorresponds to variant rs28934590dbSNPEnsembl.1
Natural variantiVAR_008546464G → R in C2D. 1 PublicationCorresponds to variant rs151340617dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi717.
MalaCardsiC2.
MIMi217000. phenotype.
603075. phenotype.
OpenTargetsiENSG00000166278.
ENSG00000204364.
ENSG00000206372.
ENSG00000226560.
ENSG00000231543.
ENSG00000235017.
ENSG00000235696.
Orphaneti169147. Immunodeficiency due to an early component of complement deficiency.
PharmGKBiPA25637.

Polymorphism and mutation databases

DMDMi3915642.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 202 PublicationsAdd BLAST20
ChainiPRO_000002761021 – 752Complement C2Add BLAST732
ChainiPRO_000002761121 – 243Complement C2b fragmentAdd BLAST223
ChainiPRO_0000027612244 – 752Complement C2a fragmentAdd BLAST509

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi24 ↔ 64
Glycosylationi29N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi51 ↔ 84
Disulfide bondi89 ↔ 131
Glycosylationi112N-linked (GlcNAc...)2 Publications1
Disulfide bondi117 ↔ 144
Disulfide bondi151 ↔ 191
Disulfide bondi177 ↔ 204
Glycosylationi290N-linked (GlcNAc...)Sequence analysis1
Glycosylationi333N-linked (GlcNAc...)3 Publications1
Glycosylationi467N-linked (GlcNAc...)2 Publications1
Glycosylationi471N-linked (GlcNAc...)1 Publication1
Disulfide bondi492 ↔ 508By similarity
Glycosylationi621N-linked (GlcNAc...)2 Publications1
Glycosylationi651N-linked (GlcNAc...) (complex)1 Publication1
Disulfide bondi675 ↔ 705By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP06681.
PeptideAtlasiP06681.
PRIDEiP06681.

PTM databases

iPTMnetiP06681.
PhosphoSitePlusiP06681.

Miscellaneous databases

PMAP-CutDBP06681.

Expressioni

Gene expression databases

BgeeiENSG00000166278.
CleanExiHS_C2.
ExpressionAtlasiP06681. baseline and differential.
GenevisibleiP06681. HS.

Organism-specific databases

HPAiCAB016775.

Interactioni

Subunit structurei

C2a interacts with Schistosoma haematobium TOR (via N-terminal extracellular domain). This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection.1 Publication

Protein-protein interaction databases

BioGridi107178. 2 interactors.
IntActiP06681. 2 interactors.
STRINGi9606.ENSP00000299367.

Structurei

Secondary structure

1752
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi34 – 39Combined sources6
Beta strandi46 – 50Combined sources5
Beta strandi55 – 59Combined sources5
Beta strandi61 – 64Combined sources4
Beta strandi83 – 86Combined sources4
Beta strandi88 – 90Combined sources3
Beta strandi98 – 102Combined sources5
Beta strandi105 – 108Combined sources4
Beta strandi112 – 117Combined sources6
Beta strandi122 – 125Combined sources4
Beta strandi127 – 131Combined sources5
Beta strandi137 – 139Combined sources3
Beta strandi143 – 145Combined sources3
Beta strandi149 – 151Combined sources3
Beta strandi160 – 163Combined sources4
Beta strandi172 – 177Combined sources6
Beta strandi182 – 185Combined sources4
Beta strandi187 – 191Combined sources5
Beta strandi197 – 199Combined sources3
Beta strandi203 – 205Combined sources3
Helixi207 – 211Combined sources5
Beta strandi249 – 260Combined sources12
Helixi267 – 284Combined sources18
Turni285 – 287Combined sources3
Beta strandi291 – 306Combined sources16
Helixi311 – 314Combined sources4
Helixi316 – 324Combined sources9
Helixi328 – 331Combined sources4
Helixi339 – 357Combined sources19
Beta strandi359 – 361Combined sources3
Helixi362 – 365Combined sources4
Beta strandi367 – 375Combined sources9
Beta strandi381 – 383Combined sources3
Helixi386 – 395Combined sources10
Helixi403 – 405Combined sources3
Beta strandi406 – 413Combined sources8
Helixi420 – 426Combined sources7
Beta strandi436 – 440Combined sources5
Helixi442 – 451Combined sources10
Helixi474 – 477Combined sources4
Beta strandi481 – 485Combined sources5
Beta strandi492 – 504Combined sources13
Helixi506 – 509Combined sources4
Helixi515 – 517Combined sources3
Beta strandi519 – 522Combined sources4
Beta strandi531 – 533Combined sources3
Beta strandi535 – 540Combined sources6
Helixi546 – 552Combined sources7
Beta strandi563 – 569Combined sources7
Beta strandi574 – 576Combined sources3
Helixi586 – 591Combined sources6
Helixi600 – 607Combined sources8
Beta strandi610 – 618Combined sources9
Beta strandi623 – 630Combined sources8
Helixi632 – 639Combined sources8
Helixi640 – 644Combined sources5
Helixi655 – 657Combined sources3
Beta strandi663 – 666Combined sources4
Helixi676 – 678Combined sources3
Beta strandi682 – 687Combined sources6
Beta strandi690 – 701Combined sources12
Turni704 – 707Combined sources4
Beta strandi720 – 723Combined sources4
Beta strandi727 – 731Combined sources5
Helixi732 – 735Combined sources4
Helixi736 – 743Combined sources8
Turni744 – 746Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2I6QX-ray2.10A244-752[»]
2I6SX-ray2.70A244-752[»]
2ODPX-ray1.90A244-752[»]
2ODQX-ray2.30A244-752[»]
3ERBX-ray1.80A21-243[»]
ProteinModelPortaliP06681.
SMRiP06681.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP06681.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini22 – 86Sushi 1PROSITE-ProRule annotationAdd BLAST65
Domaini87 – 146Sushi 2PROSITE-ProRule annotationAdd BLAST60
Domaini149 – 206Sushi 3PROSITE-ProRule annotationAdd BLAST58
Domaini254 – 452VWFAPROSITE-ProRule annotationAdd BLAST199
Domaini464 – 744Peptidase S1PROSITE-ProRule annotationAdd BLAST281

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi260 – 264MIDAS-like motif5

Domaini

The MIDAS-like motif in the VWFA domain binds divalent metal cations.

Sequence similaritiesi

Belongs to the peptidase S1 family.PROSITE-ProRule annotation
Contains 1 peptidase S1 domain.PROSITE-ProRule annotation
Contains 3 Sushi (CCP/SCR) domains.PROSITE-ProRule annotation
Contains 1 VWFA domain.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiKOG3627. Eukaryota.
COG5640. LUCA.
GeneTreeiENSGT00530000063826.
HOGENOMiHOG000038034.
HOVERGENiHBG002567.
InParanoidiP06681.
KOiK01332.
OMAiINLFRMQ.
OrthoDBiEOG091G02G8.
PhylomeDBiP06681.
TreeFamiTF330194.

Family and domain databases

CDDicd00033. CCP. 2 hits.
cd00190. Tryp_SPc. 1 hit.
Gene3Di3.40.50.410. 1 hit.
InterProiIPR011360. Compl_C2_B.
IPR009003. Peptidase_S1_PA.
IPR001314. Peptidase_S1A.
IPR000436. Sushi_SCR_CCP_dom.
IPR001254. Trypsin_dom.
IPR018114. TRYPSIN_HIS.
IPR033116. TRYPSIN_SER.
IPR002035. VWF_A.
[Graphical view]
PfamiPF00084. Sushi. 2 hits.
PF00089. Trypsin. 1 hit.
PF00092. VWA. 1 hit.
[Graphical view]
PIRSFiPIRSF001154. Compl_C2_B. 1 hit.
PRINTSiPR00722. CHYMOTRYPSIN.
SMARTiSM00032. CCP. 3 hits.
SM00020. Tryp_SPc. 1 hit.
SM00327. VWA. 1 hit.
[Graphical view]
SUPFAMiSSF50494. SSF50494. 1 hit.
SSF53300. SSF53300. 1 hit.
SSF57535. SSF57535. 3 hits.
PROSITEiPS50923. SUSHI. 3 hits.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
PS50234. VWFA. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P06681-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MGPLMVLFCL LFLYPGLADS APSCPQNVNI SGGTFTLSHG WAPGSLLTYS
60 70 80 90 100
CPQGLYPSPA SRLCKSSGQW QTPGATRSLS KAVCKPVRCP APVSFENGIY
110 120 130 140 150
TPRLGSYPVG GNVSFECEDG FILRGSPVRQ CRPNGMWDGE TAVCDNGAGH
160 170 180 190 200
CPNPGISLGA VRTGFRFGHG DKVRYRCSSN LVLTGSSERE CQGNGVWSGT
210 220 230 240 250
EPICRQPYSY DFPEDVAPAL GTSFSHMLGA TNPTQKTKES LGRKIQIQRS
260 270 280 290 300
GHLNLYLLLD CSQSVSENDF LIFKESASLM VDRIFSFEIN VSVAIITFAS
310 320 330 340 350
EPKVLMSVLN DNSRDMTEVI SSLENANYKD HENGTGTNTY AALNSVYLMM
360 370 380 390 400
NNQMRLLGME TMAWQEIRHA IILLTDGKSN MGGSPKTAVD HIREILNINQ
410 420 430 440 450
KRNDYLDIYA IGVGKLDVDW RELNELGSKK DGERHAFILQ DTKALHQVFE
460 470 480 490 500
HMLDVSKLTD TICGVGNMSA NASDQERTPW HVTIKPKSQE TCRGALISDQ
510 520 530 540 550
WVLTAAHCFR DGNDHSLWRV NVGDPKSQWG KEFLIEKAVI SPGFDVFAKK
560 570 580 590 600
NQGILEFYGD DIALLKLAQK VKMSTHARPI CLPCTMEANL ALRRPQGSTC
610 620 630 640 650
RDHENELLNK QSVPAHFVAL NGSKLNINLK MGVEWTSCAE VVSQEKTMFP
660 670 680 690 700
NLTDVREVVT DQFLCSGTQE DESPCKGESG GAVFLERRFR FFQVGLVSWG
710 720 730 740 750
LYNPCLGSAD KNSRKRAPRS KVPPPRDFHI NLFRMQPWLR QHLGDVLNFL

PL
Length:752
Mass (Da):83,268
Last modified:December 15, 1998 - v2
Checksum:i5A96A13E700CF444
GO
Isoform 2 (identifier: P06681-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-147: MGPLMVLFCL...DGETAVCDNG → MRALCIRETCSSELGFSRNWSRRK
     238-328: Missing.

Note: No experimental confirmation available.
Show »
Length:538
Mass (Da):60,318
Checksum:iDAD9613AAAF483E8
GO
Isoform 3 (identifier: P06681-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     16-147: Missing.

Note: No experimental confirmation available.
Show »
Length:620
Mass (Da):69,444
Checksum:iFC295E99940BE04D
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti30I → L AA sequence (PubMed:6922702).Curated1
Sequence conflicti34T → S AA sequence (PubMed:6922702).Curated1
Sequence conflicti211D → G in BAG62532 (PubMed:14702039).Curated1
Sequence conflicti249R → S AA sequence (PubMed:6922702).Curated1
Sequence conflicti253L → K AA sequence (PubMed:6922702).Curated1

Polymorphismi

The variant Asp-318 is associated with a reduced risk of age-related macular degeneration (ARMD) [MIMi:603075]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world.1 Publication

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_008544131C → Y in C2D. 1 PublicationCorresponds to variant rs760744400dbSNPEnsembl.1
Natural variantiVAR_008545209S → F in C2D. 1 PublicationCorresponds to variant rs28934590dbSNPEnsembl.1
Natural variantiVAR_019158318E → D.2 PublicationsCorresponds to variant rs9332739dbSNPEnsembl.1
Natural variantiVAR_008546464G → R in C2D. 1 PublicationCorresponds to variant rs151340617dbSNPEnsembl.1
Natural variantiVAR_011772533F → L.Corresponds to variant rs1042664dbSNPEnsembl.1
Natural variantiVAR_019159734R → C.1 PublicationCorresponds to variant rs4151648dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0430381 – 147MGPLM…VCDNG → MRALCIRETCSSELGFSRNW SRRK in isoform 2. 1 PublicationAdd BLAST147
Alternative sequenceiVSP_04610316 – 147Missing in isoform 3. 1 PublicationAdd BLAST132
Alternative sequenceiVSP_043039238 – 328Missing in isoform 2. 1 PublicationAdd BLAST91

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X04481 mRNA. Translation: CAA28169.1.
M26301 mRNA. Translation: AAA35614.1.
L09708, L09706, L09707 Genomic DNA. Translation: AAB97607.1.
AF019413 Genomic DNA. Translation: AAB67975.1.
AY349611 Genomic DNA. Translation: AAQ15273.1.
AK298311 mRNA. Translation: BAG60565.1.
AK300892 mRNA. Translation: BAG62532.1.
AL645922 Genomic DNA. No translation available.
AL662834 Genomic DNA. No translation available.
AL662849 Genomic DNA. No translation available.
AL671762 Genomic DNA. No translation available.
AL844853 Genomic DNA. No translation available.
BX005143 Genomic DNA. No translation available.
CR388219 Genomic DNA. No translation available.
CR759782 Genomic DNA. No translation available.
CR759784 Genomic DNA. No translation available.
CR933857 Genomic DNA. No translation available.
BC043484 mRNA. Translation: AAH43484.1.
M15549 Genomic DNA. Translation: AAA59649.1.
M15082 Genomic DNA. Translation: AAA59624.1.
CCDSiCCDS4728.1. [P06681-1]
CCDS54991.1. [P06681-3]
CCDS56416.1. [P06681-2]
PIRiA25971. C2HU.
RefSeqiNP_000054.2. NM_000063.5. [P06681-1]
NP_001139375.1. NM_001145903.2. [P06681-3]
NP_001171534.1. NM_001178063.2. [P06681-2]
UniGeneiHs.408903.

Genome annotation databases

EnsembliENST00000299367; ENSP00000299367; ENSG00000166278. [P06681-1]
ENST00000375510; ENSP00000364660; ENSG00000204364. [P06681-1]
ENST00000383362; ENSP00000372853; ENSG00000206372. [P06681-1]
ENST00000411803; ENSP00000402278; ENSG00000235696. [P06681-1]
ENST00000413548; ENSP00000407961; ENSG00000231543. [P06681-1]
ENST00000416252; ENSP00000405800; ENSG00000235017. [P06681-1]
ENST00000442278; ENSP00000395683; ENSG00000166278. [P06681-3]
ENST00000448206; ENSP00000392835; ENSG00000226560. [P06681-1]
ENST00000452323; ENSP00000392322; ENSG00000166278. [P06681-2]
ENST00000548973; ENSP00000446728; ENSG00000206372. [P06681-3]
ENST00000548995; ENSP00000449286; ENSG00000204364. [P06681-3]
ENST00000549972; ENSP00000447632; ENSG00000235696. [P06681-3]
ENST00000550682; ENSP00000446639; ENSG00000231543. [P06681-3]
ENST00000551081; ENSP00000450387; ENSG00000235017. [P06681-3]
ENST00000551648; ENSP00000449715; ENSG00000226560. [P06681-3]
ENST00000612228; ENSP00000482616; ENSG00000231543. [P06681-2]
ENST00000615380; ENSP00000481651; ENSG00000226560. [P06681-2]
ENST00000618254; ENSP00000483231; ENSG00000235017. [P06681-2]
ENST00000621558; ENSP00000480739; ENSG00000206372. [P06681-2]
GeneIDi717.
KEGGihsa:717.
UCSCiuc010jtk.5. human. [P06681-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Web resourcesi

C2base

C2 mutation db

SeattleSNPs

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X04481 mRNA. Translation: CAA28169.1.
M26301 mRNA. Translation: AAA35614.1.
L09708, L09706, L09707 Genomic DNA. Translation: AAB97607.1.
AF019413 Genomic DNA. Translation: AAB67975.1.
AY349611 Genomic DNA. Translation: AAQ15273.1.
AK298311 mRNA. Translation: BAG60565.1.
AK300892 mRNA. Translation: BAG62532.1.
AL645922 Genomic DNA. No translation available.
AL662834 Genomic DNA. No translation available.
AL662849 Genomic DNA. No translation available.
AL671762 Genomic DNA. No translation available.
AL844853 Genomic DNA. No translation available.
BX005143 Genomic DNA. No translation available.
CR388219 Genomic DNA. No translation available.
CR759782 Genomic DNA. No translation available.
CR759784 Genomic DNA. No translation available.
CR933857 Genomic DNA. No translation available.
BC043484 mRNA. Translation: AAH43484.1.
M15549 Genomic DNA. Translation: AAA59649.1.
M15082 Genomic DNA. Translation: AAA59624.1.
CCDSiCCDS4728.1. [P06681-1]
CCDS54991.1. [P06681-3]
CCDS56416.1. [P06681-2]
PIRiA25971. C2HU.
RefSeqiNP_000054.2. NM_000063.5. [P06681-1]
NP_001139375.1. NM_001145903.2. [P06681-3]
NP_001171534.1. NM_001178063.2. [P06681-2]
UniGeneiHs.408903.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2I6QX-ray2.10A244-752[»]
2I6SX-ray2.70A244-752[»]
2ODPX-ray1.90A244-752[»]
2ODQX-ray2.30A244-752[»]
3ERBX-ray1.80A21-243[»]
ProteinModelPortaliP06681.
SMRiP06681.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107178. 2 interactors.
IntActiP06681. 2 interactors.
STRINGi9606.ENSP00000299367.

Protein family/group databases

MEROPSiS01.194.

PTM databases

iPTMnetiP06681.
PhosphoSitePlusiP06681.

Polymorphism and mutation databases

DMDMi3915642.

Proteomic databases

PaxDbiP06681.
PeptideAtlasiP06681.
PRIDEiP06681.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000299367; ENSP00000299367; ENSG00000166278. [P06681-1]
ENST00000375510; ENSP00000364660; ENSG00000204364. [P06681-1]
ENST00000383362; ENSP00000372853; ENSG00000206372. [P06681-1]
ENST00000411803; ENSP00000402278; ENSG00000235696. [P06681-1]
ENST00000413548; ENSP00000407961; ENSG00000231543. [P06681-1]
ENST00000416252; ENSP00000405800; ENSG00000235017. [P06681-1]
ENST00000442278; ENSP00000395683; ENSG00000166278. [P06681-3]
ENST00000448206; ENSP00000392835; ENSG00000226560. [P06681-1]
ENST00000452323; ENSP00000392322; ENSG00000166278. [P06681-2]
ENST00000548973; ENSP00000446728; ENSG00000206372. [P06681-3]
ENST00000548995; ENSP00000449286; ENSG00000204364. [P06681-3]
ENST00000549972; ENSP00000447632; ENSG00000235696. [P06681-3]
ENST00000550682; ENSP00000446639; ENSG00000231543. [P06681-3]
ENST00000551081; ENSP00000450387; ENSG00000235017. [P06681-3]
ENST00000551648; ENSP00000449715; ENSG00000226560. [P06681-3]
ENST00000612228; ENSP00000482616; ENSG00000231543. [P06681-2]
ENST00000615380; ENSP00000481651; ENSG00000226560. [P06681-2]
ENST00000618254; ENSP00000483231; ENSG00000235017. [P06681-2]
ENST00000621558; ENSP00000480739; ENSG00000206372. [P06681-2]
GeneIDi717.
KEGGihsa:717.
UCSCiuc010jtk.5. human. [P06681-1]

Organism-specific databases

CTDi717.
DisGeNETi717.
GeneCardsiC2.
HGNCiHGNC:1248. C2.
HPAiCAB016775.
MalaCardsiC2.
MIMi217000. phenotype.
603075. phenotype.
613927. gene.
neXtProtiNX_P06681.
OpenTargetsiENSG00000166278.
ENSG00000204364.
ENSG00000206372.
ENSG00000226560.
ENSG00000231543.
ENSG00000235017.
ENSG00000235696.
Orphaneti169147. Immunodeficiency due to an early component of complement deficiency.
PharmGKBiPA25637.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3627. Eukaryota.
COG5640. LUCA.
GeneTreeiENSGT00530000063826.
HOGENOMiHOG000038034.
HOVERGENiHBG002567.
InParanoidiP06681.
KOiK01332.
OMAiINLFRMQ.
OrthoDBiEOG091G02G8.
PhylomeDBiP06681.
TreeFamiTF330194.

Enzyme and pathway databases

BioCyciZFISH:HS09368-MONOMER.
ReactomeiR-HSA-166663. Initial triggering of complement.
R-HSA-174577. Activation of C3 and C5.
R-HSA-977606. Regulation of Complement cascade.
SABIO-RKP06681.

Miscellaneous databases

ChiTaRSiC2. human.
EvolutionaryTraceiP06681.
GeneWikiiComplement_component_2.
GenomeRNAii717.
PMAP-CutDBP06681.
PROiP06681.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000166278.
CleanExiHS_C2.
ExpressionAtlasiP06681. baseline and differential.
GenevisibleiP06681. HS.

Family and domain databases

CDDicd00033. CCP. 2 hits.
cd00190. Tryp_SPc. 1 hit.
Gene3Di3.40.50.410. 1 hit.
InterProiIPR011360. Compl_C2_B.
IPR009003. Peptidase_S1_PA.
IPR001314. Peptidase_S1A.
IPR000436. Sushi_SCR_CCP_dom.
IPR001254. Trypsin_dom.
IPR018114. TRYPSIN_HIS.
IPR033116. TRYPSIN_SER.
IPR002035. VWF_A.
[Graphical view]
PfamiPF00084. Sushi. 2 hits.
PF00089. Trypsin. 1 hit.
PF00092. VWA. 1 hit.
[Graphical view]
PIRSFiPIRSF001154. Compl_C2_B. 1 hit.
PRINTSiPR00722. CHYMOTRYPSIN.
SMARTiSM00032. CCP. 3 hits.
SM00020. Tryp_SPc. 1 hit.
SM00327. VWA. 1 hit.
[Graphical view]
SUPFAMiSSF50494. SSF50494. 1 hit.
SSF53300. SSF53300. 1 hit.
SSF57535. SSF57535. 3 hits.
PROSITEiPS50923. SUSHI. 3 hits.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
PS50234. VWFA. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCO2_HUMAN
AccessioniPrimary (citable) accession number: P06681
Secondary accession number(s): B4DPF3
, B4DV20, E9PFN7, O19694, Q13904
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: December 15, 1998
Last modified: November 30, 2016
This is version 192 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

Miscellaneous

C2 is a major histocompatibility complex class-III protein.

Keywords - Technical termi

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

Documents

  1. Human chromosome 6
    Human chromosome 6: 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. Peptidase families
    Classification of peptidase families and list of entries
  7. 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.