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Protein

Complement C2

Gene

C2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript 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)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi269 – 2691Divalent metal cationBy similarity
Metal bindingi271 – 2711Divalent metal cationBy similarity
Metal bindingi344 – 3441Divalent metal cationBy similarity
Active sitei514 – 5141Charge relay systemBy similarity
Active sitei570 – 5701Charge relay systemBy similarity
Active sitei689 – 6891Charge relay systemBy similarity

GO - Molecular functioni

  1. metal ion binding Source: UniProtKB-KW
  2. serine-type endopeptidase activity Source: InterPro

GO - Biological processi

  1. complement activation Source: MGI
  2. complement activation, classical pathway Source: UniProtKB-KW
  3. innate immune response Source: UniProtKB-KW
  4. positive regulation of apoptotic cell clearance Source: MGI
  5. 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

ReactomeiREACT_198562. Regulation of Complement cascade.
REACT_202834. Initial triggering of complement.
REACT_213087. Activation of C3 and C5.

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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 17

Organism-specific databases

MGIiMGI:88226. C2.

Subcellular locationi

GO - Cellular componenti

  1. extracellular vesicular exosome Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Secreted

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 1818Add
BLAST
Chaini19 – 760742Complement C2PRO_0000027613Add
BLAST
Chaini19 – 250232Complement C2b fragmentPRO_0000027614Add
BLAST
Chaini251 – 760510Complement C2a fragmentPRO_0000027615Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi22 ↔ 62By similarity
Glycosylationi27 – 271N-linked (GlcNAc...)Sequence Analysis
Glycosylationi32 – 321N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi49 ↔ 89By similarity
Disulfide bondi94 ↔ 136By similarity
Glycosylationi117 – 1171N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi122 ↔ 149By similarity
Disulfide bondi156 ↔ 197By similarity
Disulfide bondi182 ↔ 210By similarity
Glycosylationi297 – 2971N-linked (GlcNAc...)Sequence Analysis
Glycosylationi340 – 3401N-linked (GlcNAc...)Sequence Analysis
Glycosylationi474 – 4741N-linked (GlcNAc...)Sequence Analysis
Glycosylationi478 – 4781N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi499 ↔ 515By similarity
Glycosylationi663 – 6631N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi685 ↔ 715By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP21180.
PRIDEiP21180.

PTM databases

PhosphoSiteiP21180.

Expressioni

Gene expression databases

BgeeiP21180.
CleanExiMM_C2.
ExpressionAtlasiP21180. baseline and differential.
GenevestigatoriP21180.

Structurei

3D structure databases

ProteinModelPortaliP21180.
SMRiP21180. Positions 22-223, 251-760.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini20 – 9071Sushi 1PROSITE-ProRule annotationAdd
BLAST
Domaini92 – 15160Sushi 2PROSITE-ProRule annotationAdd
BLAST
Domaini154 – 21259Sushi 3PROSITE-ProRule annotationAdd
BLAST
Domaini261 – 459199VWFAPROSITE-ProRule annotationAdd
BLAST
Domaini471 – 752282Peptidase S1PROSITE-ProRule annotationAdd
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi267 – 2715MIDAS-like motifBy similarity

Domaini

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

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

GeneTreeiENSGT00530000063826.
HOGENOMiHOG000038034.
HOVERGENiHBG002567.
InParanoidiP21180.
KOiK01332.
OMAiRDMTEVI.
TreeFamiTF330194.

Family and domain databases

Gene3Di3.40.50.410. 1 hit.
InterProiIPR011360. Compl_C2_B.
IPR001254. Peptidase_S1.
IPR018114. Peptidase_S1_AS.
IPR001314. Peptidase_S1A.
IPR000436. Sushi_SCR_CCP_dom.
IPR009003. Trypsin-like_Pept_dom.
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. 2 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 (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. Align

Isoform Long (identifier: P21180-1) [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
MAPLLALFYL LQLGPGLAAL FCNQNVNITG GNFTLSHGWA PGSLLIYSCP
60 70 80 90 100
LGRYPSPAWR KCQSNGQWLT PRSSSHHTLR SSRMVKAVCK PVRCLAPSSF
110 120 130 140 150
ENGIYFPRLV SYPVGSNVSF ECEQDFTLRG SPVRYCRPNG LWDGETAVCD
160 170 180 190 200
NGASHCPNPG ISVGTARTGL NFDLGDKVRY RCSSSNMVLT GSAERECQSN
210 220 230 240 250
GVWSGSEPIC RQPYSYDFPE DVASALDTSL TNLLGATNPT QNLLTKSLGR
260 270 280 290 300
KIIIQRSGHL NLYLLLDASQ SVTEKDFDIF KKSAELMVER IFSFEVNVSV
310 320 330 340 350
AIITFASQPK TIMSILSERS QDVTEVITSL DSASYKDHEN ATGTNTYEVL
360 370 380 390 400
IRVYSMMQSQ MDRLGMETSA WKEIRHTIIL LTDGKSNMGD SPKKAVTRIR
410 420 430 440 450
ELLSIEQNRD DYLDIYAIGV GKLDVDWKEL NELGSKKDGE RHAFILQDAK
460 470 480 490 500
ALQQIFEHML DVSKLTDTIC GVGNMSANAS DQERTPWQVT FKPKSKETCQ
510 520 530 540 550
GSLISDQWVL TAAHCFHDIQ MEDHHLWRVN VGDPTSQHGK EFLVEDVIIA
560 570 580 590 600
PGFNVHAKRK QGISEFYADD IALLKLSRKV KMSTHARPIC LPCTVGANMA
610 620 630 640 650
LRRSPGSTCK DHETELLSQQ KVPAHFVALN GNRLNINLRT GPEWTRCIQA
660 670 680 690 700
VSQNKNIFPS LTNVSEVVTD QFLCSGMEEE DDNPCKGESG GAVFLGRRYR
710 720 730 740 750
FFQVGLVSWG LFDPCHGSSN KNLRKKPPRG VLPRDFHISL FRLQPWLRQH
760
LDGVLDFLPL
Length:760
Mass (Da):84,741
Last modified:July 27, 2011 - v2
Checksum:i91C896A3EDC7D448
GO
Isoform Short (identifier: P21180-2) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     606-612: Missing.

Show »
Length:753
Mass (Da):84,013
Checksum:iC997F5CBA3A12278
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti123 – 1242EQ → DE in AAA37380. (PubMed:2229060)Curated
Sequence conflicti123 – 1242EQ → DE in AAA37381. (PubMed:2229060)Curated
Sequence conflicti123 – 1242EQ → DE in AAA63294. (PubMed:2229060)Curated
Sequence conflicti299 – 2991S → T in AAA37380. (PubMed:2229060)Curated
Sequence conflicti299 – 2991S → T in AAA37381. (PubMed:2229060)Curated
Sequence conflicti299 – 2991S → T in AAA63294. (PubMed:2229060)Curated
Sequence conflicti344 – 3441T → A in AAA37380. (PubMed:2229060)Curated
Sequence conflicti344 – 3441T → A in AAA37381. (PubMed:2229060)Curated
Sequence conflicti344 – 3441T → A in AAA63294. (PubMed:2229060)Curated
Sequence conflicti359 – 3591S → T in AAA37380. (PubMed:2229060)Curated
Sequence conflicti359 – 3591S → T in AAA37381. (PubMed:2229060)Curated
Sequence conflicti359 – 3591S → T in AAA63294. (PubMed:2229060)Curated

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei606 – 6127Missing in isoform Short. 1 PublicationVSP_005385

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M57891 mRNA. Translation: AAA63294.1.
M60579
, M60563, M60564, M60565, M60566, M60567, M60568, M60569, M60570, M60571, M60572, M60573, M60574, M60575, M60605, M60576, M60577, M60578 Genomic DNA. Translation: AAA37380.1.
M60579
, M60563, M60564, M60565, M60566, M60567, M60568, M60569, M60570, M60571, M60572, M60573, M60574, M60575, M60605, M60576, M60577, M60578 Genomic DNA. Translation: AAA37381.1.
AK146812 mRNA. Translation: BAE27452.1.
AF109906 Genomic DNA. Translation: AAC84162.1.
CT025759 Genomic DNA. Translation: CAX15332.1.
CH466666 Genomic DNA. Translation: EDL26734.1.
BC011086 mRNA. Translation: AAH11086.1.
CCDSiCCDS28664.1. [P21180-1]
PIRiA38876. C2MS.
RefSeqiNP_038512.2. NM_013484.2. [P21180-1]
UniGeneiMm.283217.

Genome annotation databases

EnsembliENSMUST00000025230; ENSMUSP00000025230; ENSMUSG00000024371. [P21180-1]
GeneIDi12263.
KEGGimmu:12263.
UCSCiuc008cdz.2. mouse. [P21180-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M57891 mRNA. Translation: AAA63294.1.
M60579
, M60563, M60564, M60565, M60566, M60567, M60568, M60569, M60570, M60571, M60572, M60573, M60574, M60575, M60605, M60576, M60577, M60578 Genomic DNA. Translation: AAA37380.1.
M60579
, M60563, M60564, M60565, M60566, M60567, M60568, M60569, M60570, M60571, M60572, M60573, M60574, M60575, M60605, M60576, M60577, M60578 Genomic DNA. Translation: AAA37381.1.
AK146812 mRNA. Translation: BAE27452.1.
AF109906 Genomic DNA. Translation: AAC84162.1.
CT025759 Genomic DNA. Translation: CAX15332.1.
CH466666 Genomic DNA. Translation: EDL26734.1.
BC011086 mRNA. Translation: AAH11086.1.
CCDSiCCDS28664.1. [P21180-1]
PIRiA38876. C2MS.
RefSeqiNP_038512.2. NM_013484.2. [P21180-1]
UniGeneiMm.283217.

3D structure databases

ProteinModelPortaliP21180.
SMRiP21180. Positions 22-223, 251-760.
ModBaseiSearch...
MobiDBiSearch...

Protein family/group databases

MEROPSiS01.194.

PTM databases

PhosphoSiteiP21180.

Proteomic databases

MaxQBiP21180.
PRIDEiP21180.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000025230; ENSMUSP00000025230; ENSMUSG00000024371. [P21180-1]
GeneIDi12263.
KEGGimmu:12263.
UCSCiuc008cdz.2. mouse. [P21180-1]

Organism-specific databases

CTDi717.
MGIiMGI:88226. C2.

Phylogenomic databases

GeneTreeiENSGT00530000063826.
HOGENOMiHOG000038034.
HOVERGENiHBG002567.
InParanoidiP21180.
KOiK01332.
OMAiRDMTEVI.
TreeFamiTF330194.

Enzyme and pathway databases

ReactomeiREACT_198562. Regulation of Complement cascade.
REACT_202834. Initial triggering of complement.
REACT_213087. Activation of C3 and C5.

Miscellaneous databases

ChiTaRSiC2. mouse.
NextBioi280706.
PROiP21180.
SOURCEiSearch...

Gene expression databases

BgeeiP21180.
CleanExiMM_C2.
ExpressionAtlasiP21180. baseline and differential.
GenevestigatoriP21180.

Family and domain databases

Gene3Di3.40.50.410. 1 hit.
InterProiIPR011360. Compl_C2_B.
IPR001254. Peptidase_S1.
IPR018114. Peptidase_S1_AS.
IPR001314. Peptidase_S1A.
IPR000436. Sushi_SCR_CCP_dom.
IPR009003. Trypsin-like_Pept_dom.
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. 2 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...

Publicationsi

« Hide 'large scale' publications
  1. "Murine complement C2 and factor B genomic and cDNA cloning reveals different mechanisms for multiple transcripts of C2 and B."
    Ishikawa N., Nonaka M., Wetsel R.A., Colten H.R.
    J. Biol. Chem. 265:19040-19046(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORMS LONG AND SHORT).
  2. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6J.
    Tissue: Amnion.
  3. "Analysis of the gene-dense major histocompatibility complex class III region and its comparison to mouse."
    Xie T., Rowen L., Aguado B., Ahearn M.E., Madan A., Qin S., Campbell R.D., Hood L.
    Genome Res. 13:2621-2636(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    Strain: 129.
  4. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    Strain: C57BL/6J.
  5. Mural R.J., Adams M.D., Myers E.W., Smith H.O., Venter J.C.
    Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Mammary tumor.

Entry informationi

Entry nameiCO2_MOUSE
AccessioniPrimary (citable) accession number: P21180
Secondary accession number(s): O70350
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 1, 1991
Last sequence update: July 27, 2011
Last modified: February 4, 2015
This is version 133 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

C2 is a major histocompatibility complex class-III protein.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.