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UniProtKB - P06681 (CO2_HUMAN)

Protein

Complement C2

Gene

C2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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.

Miscellaneous

C2 is a major histocompatibility complex class-III protein.

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

View the complete GO annotation on QuickGO ...

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
View the complete GO annotation on QuickGO ...

Keywordsi

Molecular functionHydrolase, Protease, Serine protease
Biological processComplement pathway, Immunity, Innate immunity
LigandMetal-binding

Enzyme and pathway databases

ReactomeiR-HSA-166663 Initial triggering of complement
R-HSA-174577 Activation of C3 and C5
R-HSA-977606 Regulation of Complement cascade
SABIO-RKiP06681

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:
Complement C2b fragment
Complement C2a fragment
Gene namesi
Name:C2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

EuPathDBiHostDB:ENSG00000166278.14
HGNCiHGNC:1248 C2
MIMi613927 gene
neXtProtiNX_P06681

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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 dbSNP:rs760744400Ensembl.1
Natural variantiVAR_008545209S → F in C2D. 1 PublicationCorresponds to variant dbSNP:rs28934590Ensembl.1
Natural variantiVAR_008546464G → R in C2D. 1 PublicationCorresponds to variant dbSNP:rs151340617Ensembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi717
MalaCardsiC2
MIMi217000 phenotype
603075 phenotype
OpenTargetsiENSG00000166278
Orphaneti169147 Immunodeficiency due to a classical component pathway 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...) asparagineSequence analysis1
Disulfide bondi51 ↔ 84
Disulfide bondi89 ↔ 131
Glycosylationi112N-linked (GlcNAc...) asparagine2 Publications1
Disulfide bondi117 ↔ 144
Disulfide bondi151 ↔ 191
Disulfide bondi177 ↔ 204
Glycosylationi290N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi333N-linked (GlcNAc...) asparagine3 Publications1
Glycosylationi467N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi471N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi492 ↔ 508By similarity
Glycosylationi621N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi651N-linked (GlcNAc...) (complex) asparagine1 Publication1
Disulfide bondi675 ↔ 705By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP06681
PeptideAtlasiP06681
PRIDEiP06681
ProteomicsDBi51908
51909 [P06681-2]

PTM databases

GlyConnecti1145
iPTMnetiP06681
PhosphoSitePlusiP06681

Miscellaneous databases

PMAP-CutDBiP06681

Expressioni

Gene expression databases

BgeeiENSG00000166278 Expressed in 96 organ(s), highest expression level in right lobe of liver
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

Binary interactionsi

WithEntry#Exp.IntActNotes
CREB3O43889-23EBI-2835920,EBI-625022

Protein-protein interaction databases

BioGridi107178, 2 interactors
IntActiP06681, 11 interactors
STRINGi9606.ENSP00000299367

Structurei

Secondary structure

1752
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiKOG3627 Eukaryota
COG5640 LUCA
GeneTreeiENSGT00530000063826
HOGENOMiHOG000038034
HOVERGENiHBG002567
InParanoidiP06681
KOiK01332
OMAiRNVSEFY
OrthoDBiEOG093702XZ
PhylomeDBiP06681
TreeFamiTF330194

Family and domain databases

CDDicd00033 CCP, 3 hits
cd00190 Tryp_SPc, 1 hit
Gene3Di3.40.50.410, 1 hit
InterProiView protein in InterPro
IPR011360 Compl_C2_B
IPR037568 Complement_C2
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR035976 Sushi/SCR/CCP_sf
IPR000436 Sushi_SCR_CCP_dom
IPR001254 Trypsin_dom
IPR018114 TRYPSIN_HIS
IPR033116 TRYPSIN_SER
IPR002035 VWF_A
IPR036465 vWFA_dom_sf
PANTHERiPTHR19325:SF387 PTHR19325:SF387, 1 hit
PfamiView protein in Pfam
PF00084 Sushi, 2 hits
PF00089 Trypsin, 1 hit
PF00092 VWA, 1 hit
PIRSFiPIRSF001154 Compl_C2_B, 1 hit
PRINTSiPR00722 CHYMOTRYPSIN
SMARTiView protein in SMART
SM00032 CCP, 3 hits
SM00020 Tryp_SPc, 1 hit
SM00327 VWA, 1 hit
SUPFAMiSSF50494 SSF50494, 1 hit
SSF53300 SSF53300, 1 hit
SSF57535 SSF57535, 3 hits
PROSITEiView protein in PROSITE
PS50923 SUSHI, 3 hits
PS50240 TRYPSIN_DOM, 1 hit
PS00134 TRYPSIN_HIS, 1 hit
PS00135 TRYPSIN_SER, 1 hit
PS50234 VWFA, 1 hit

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

This entry has 3 described isoforms and 18 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 18 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A0G2JL69A0A0G2JL69_HUMAN
Complement C2
C2
723Annotation score:
H0Y3H6H0Y3H6_HUMAN
Complement C2
C2
526Annotation score:
A0A0G2JIE7A0A0G2JIE7_HUMAN
Complement C2
C2
525Annotation score:
Q8N6L6Q8N6L6_HUMAN
C2 protein
C2
328Annotation score:
F2Z3N2F2Z3N2_HUMAN
Complement C2
C2
203Annotation score:
A0A0G2JK28A0A0G2JK28_HUMAN
Complement C2
C2
353Annotation score:
Q5ST52Q5ST52_HUMAN
Complement C2
C2
139Annotation score:
F8WCJ9F8WCJ9_HUMAN
Complement C2
C2
104Annotation score:
C9JYQ5C9JYQ5_HUMAN
Complement C2
C2
136Annotation score:
A2ABG0A2ABG0_HUMAN
Complement C2
C2
158Annotation score:
There are more potential isoformsShow all

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 dbSNP:rs760744400Ensembl.1
Natural variantiVAR_008545209S → F in C2D. 1 PublicationCorresponds to variant dbSNP:rs28934590Ensembl.1
Natural variantiVAR_019158318E → D2 PublicationsCorresponds to variant dbSNP:rs9332739EnsemblClinVar.1
Natural variantiVAR_008546464G → R in C2D. 1 PublicationCorresponds to variant dbSNP:rs151340617Ensembl.1
Natural variantiVAR_011772533F → L. Corresponds to variant dbSNP:rs1042664Ensembl.1
Natural variantiVAR_019159734R → C1 PublicationCorresponds to variant dbSNP:rs4151648Ensembl.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

Similar proteinsi

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, 11 interactors
STRINGi9606.ENSP00000299367

Protein family/group databases

MEROPSiS01.194

PTM databases

GlyConnecti1145
iPTMnetiP06681
PhosphoSitePlusiP06681

Polymorphism and mutation databases

DMDMi3915642

Proteomic databases

PaxDbiP06681
PeptideAtlasiP06681
PRIDEiP06681
ProteomicsDBi51908
51909 [P06681-2]

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
EuPathDBiHostDB:ENSG00000166278.14
GeneCardsiC2
HGNCiHGNC:1248 C2
HPAiCAB016775
MalaCardsiC2
MIMi217000 phenotype
603075 phenotype
613927 gene
neXtProtiNX_P06681
OpenTargetsiENSG00000166278
Orphaneti169147 Immunodeficiency due to a classical component pathway complement deficiency
PharmGKBiPA25637
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3627 Eukaryota
COG5640 LUCA
GeneTreeiENSGT00530000063826
HOGENOMiHOG000038034
HOVERGENiHBG002567
InParanoidiP06681
KOiK01332
OMAiRNVSEFY
OrthoDBiEOG093702XZ
PhylomeDBiP06681
TreeFamiTF330194

Enzyme and pathway databases

ReactomeiR-HSA-166663 Initial triggering of complement
R-HSA-174577 Activation of C3 and C5
R-HSA-977606 Regulation of Complement cascade
SABIO-RKiP06681

Miscellaneous databases

ChiTaRSiC2 human
EvolutionaryTraceiP06681
GeneWikiiComplement_component_2
GenomeRNAii717
PMAP-CutDBiP06681
PROiPR:P06681
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000166278 Expressed in 96 organ(s), highest expression level in right lobe of liver
CleanExiHS_C2
ExpressionAtlasiP06681 baseline and differential
GenevisibleiP06681 HS

Family and domain databases

CDDicd00033 CCP, 3 hits
cd00190 Tryp_SPc, 1 hit
Gene3Di3.40.50.410, 1 hit
InterProiView protein in InterPro
IPR011360 Compl_C2_B
IPR037568 Complement_C2
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR035976 Sushi/SCR/CCP_sf
IPR000436 Sushi_SCR_CCP_dom
IPR001254 Trypsin_dom
IPR018114 TRYPSIN_HIS
IPR033116 TRYPSIN_SER
IPR002035 VWF_A
IPR036465 vWFA_dom_sf
PANTHERiPTHR19325:SF387 PTHR19325:SF387, 1 hit
PfamiView protein in Pfam
PF00084 Sushi, 2 hits
PF00089 Trypsin, 1 hit
PF00092 VWA, 1 hit
PIRSFiPIRSF001154 Compl_C2_B, 1 hit
PRINTSiPR00722 CHYMOTRYPSIN
SMARTiView protein in SMART
SM00032 CCP, 3 hits
SM00020 Tryp_SPc, 1 hit
SM00327 VWA, 1 hit
SUPFAMiSSF50494 SSF50494, 1 hit
SSF53300 SSF53300, 1 hit
SSF57535 SSF57535, 3 hits
PROSITEiView protein in PROSITE
PS50923 SUSHI, 3 hits
PS50240 TRYPSIN_DOM, 1 hit
PS00134 TRYPSIN_HIS, 1 hit
PS00135 TRYPSIN_SER, 1 hit
PS50234 VWFA, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCO2_HUMAN
AccessioniPrimary (citable) accession number: P06681
Secondary accession number(s): B4DPF3
, B4DV20, E9PFN7, O19694, Q13904
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: December 15, 1998
Last modified: November 7, 2018
This is version 206 of the entry and version 2 of the sequence. See 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. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Peptidase families
    Classification of peptidase families and list of entries
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
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