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P21180

- CO2_MOUSE

UniProt

P21180 - CO2_MOUSE

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Protein

Complement C2

Gene
C2
Organism
Mus musculus (Mouse)
Status
Reviewed - 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 4b 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 cation By similarity
Metal bindingi271 – 2711Divalent metal cation By similarity
Metal bindingi344 – 3441Divalent metal cation By similarity
Active sitei514 – 5141Charge relay system By similarity
Active sitei570 – 5701Charge relay system By similarity
Active sitei689 – 6891Charge relay system By similarity

GO - Molecular functioni

  1. serine-type endopeptidase activity Source: InterPro

GO - Biological processi

  1. complement activation, classical pathway Source: UniProtKB-KW
  2. innate immune response Source: UniProtKB-KW
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 region Source: UniProtKB-SubCell
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 ↔ 62 By similarity
Glycosylationi27 – 271N-linked (GlcNAc...) Reviewed prediction
Glycosylationi32 – 321N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi49 ↔ 89 By similarity
Disulfide bondi94 ↔ 136 By similarity
Glycosylationi117 – 1171N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi122 ↔ 149 By similarity
Disulfide bondi156 ↔ 197 By similarity
Disulfide bondi182 ↔ 210 By similarity
Glycosylationi297 – 2971N-linked (GlcNAc...) Reviewed prediction
Glycosylationi340 – 3401N-linked (GlcNAc...) Reviewed prediction
Glycosylationi474 – 4741N-linked (GlcNAc...) Reviewed prediction
Glycosylationi478 – 4781N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi499 ↔ 515 By similarity
Glycosylationi663 – 6631N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi685 ↔ 715 By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP21180.
PRIDEiP21180.

PTM databases

PhosphoSiteiP21180.

Expressioni

Gene expression databases

ArrayExpressiP21180.
BgeeiP21180.
CleanExiMM_C2.
GenevestigatoriP21180.

Structurei

3D structure databases

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

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini20 – 9071Sushi 1Add
BLAST
Domaini92 – 15160Sushi 2Add
BLAST
Domaini154 – 21259Sushi 3Add
BLAST
Domaini261 – 459199VWFAAdd
BLAST
Domaini471 – 752282Peptidase S1Add
BLAST

Motif

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

Domaini

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

Sequence similaritiesi

Belongs to the peptidase S1 family.
Contains 1 VWFA domain.

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

GeneTreeiENSGT00530000063826.
HOGENOMiHOG000038034.
HOVERGENiHBG002567.
InParanoidiO70350.
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.
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

MAPLLALFYL LQLGPGLAAL FCNQNVNITG GNFTLSHGWA PGSLLIYSCP    50
LGRYPSPAWR KCQSNGQWLT PRSSSHHTLR SSRMVKAVCK PVRCLAPSSF 100
ENGIYFPRLV SYPVGSNVSF ECEQDFTLRG SPVRYCRPNG LWDGETAVCD 150
NGASHCPNPG ISVGTARTGL NFDLGDKVRY RCSSSNMVLT GSAERECQSN 200
GVWSGSEPIC RQPYSYDFPE DVASALDTSL TNLLGATNPT QNLLTKSLGR 250
KIIIQRSGHL NLYLLLDASQ SVTEKDFDIF KKSAELMVER IFSFEVNVSV 300
AIITFASQPK TIMSILSERS QDVTEVITSL DSASYKDHEN ATGTNTYEVL 350
IRVYSMMQSQ MDRLGMETSA WKEIRHTIIL LTDGKSNMGD SPKKAVTRIR 400
ELLSIEQNRD DYLDIYAIGV GKLDVDWKEL NELGSKKDGE RHAFILQDAK 450
ALQQIFEHML DVSKLTDTIC GVGNMSANAS DQERTPWQVT FKPKSKETCQ 500
GSLISDQWVL TAAHCFHDIQ MEDHHLWRVN VGDPTSQHGK EFLVEDVIIA 550
PGFNVHAKRK QGISEFYADD IALLKLSRKV KMSTHARPIC LPCTVGANMA 600
LRRSPGSTCK DHETELLSQQ KVPAHFVALN GNRLNINLRT GPEWTRCIQA 650
VSQNKNIFPS LTNVSEVVTD QFLCSGMEEE DDNPCKGESG GAVFLGRRYR 700
FFQVGLVSWG LFDPCHGSSN KNLRKKPPRG VLPRDFHISL FRLQPWLRQH 750
LDGVLDFLPL 760
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

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei606 – 6127Missing in isoform Short. VSP_005385

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti123 – 1242EQ → DE in AAA37380. 1 Publication
Sequence conflicti123 – 1242EQ → DE in AAA37381. 1 Publication
Sequence conflicti123 – 1242EQ → DE in AAA63294. 1 Publication
Sequence conflicti299 – 2991S → T in AAA37380. 1 Publication
Sequence conflicti299 – 2991S → T in AAA37381. 1 Publication
Sequence conflicti299 – 2991S → T in AAA63294. 1 Publication
Sequence conflicti344 – 3441T → A in AAA37380. 1 Publication
Sequence conflicti344 – 3441T → A in AAA37381. 1 Publication
Sequence conflicti344 – 3441T → A in AAA63294. 1 Publication
Sequence conflicti359 – 3591S → T in AAA37380. 1 Publication
Sequence conflicti359 – 3591S → T in AAA37381. 1 Publication
Sequence conflicti359 – 3591S → T in AAA63294. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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:
EMBL
GenBank
DDBJ
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 .
CCDSi CCDS28664.1. [P21180-1 ]
PIRi A38876. C2MS.
RefSeqi NP_038512.2. NM_013484.2. [P21180-1 ]
UniGenei Mm.283217.

3D structure databases

ProteinModelPortali P21180.
SMRi P21180. Positions 22-223, 251-760.
ModBasei Search...
MobiDBi Search...

Protein family/group databases

MEROPSi S01.194.

PTM databases

PhosphoSitei P21180.

Proteomic databases

MaxQBi P21180.
PRIDEi P21180.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000025230 ; ENSMUSP00000025230 ; ENSMUSG00000024371 . [P21180-1 ]
GeneIDi 12263.
KEGGi mmu:12263.
UCSCi uc008cdz.2. mouse. [P21180-1 ]

Organism-specific databases

CTDi 717.
MGIi MGI:88226. C2.

Phylogenomic databases

GeneTreei ENSGT00530000063826.
HOGENOMi HOG000038034.
HOVERGENi HBG002567.
InParanoidi O70350.
KOi K01332.
OMAi RDMTEVI.
TreeFami TF330194.

Enzyme and pathway databases

Reactomei REACT_198562. Regulation of Complement cascade.
REACT_202834. Initial triggering of complement.
REACT_213087. Activation of C3 and C5.

Miscellaneous databases

ChiTaRSi C2. mouse.
NextBioi 280706.
PROi P21180.
SOURCEi Search...

Gene expression databases

ArrayExpressi P21180.
Bgeei P21180.
CleanExi MM_C2.
Genevestigatori P21180.

Family and domain databases

Gene3Di 3.40.50.410. 1 hit.
InterProi IPR011360. Compl_C2_B.
IPR001254. Peptidase_S1.
IPR018114. Peptidase_S1_AS.
IPR001314. Peptidase_S1A.
IPR000436. Sushi_SCR_CCP.
IPR009003. Trypsin-like_Pept_dom.
IPR002035. VWF_A.
[Graphical view ]
Pfami PF00084. Sushi. 2 hits.
PF00089. Trypsin. 1 hit.
PF00092. VWA. 1 hit.
[Graphical view ]
PIRSFi PIRSF001154. Compl_C2_B. 1 hit.
PRINTSi PR00722. CHYMOTRYPSIN.
SMARTi SM00032. CCP. 2 hits.
SM00020. Tryp_SPc. 1 hit.
SM00327. VWA. 1 hit.
[Graphical view ]
SUPFAMi SSF50494. SSF50494. 1 hit.
SSF53300. SSF53300. 1 hit.
SSF57535. SSF57535. 3 hits.
PROSITEi PS50923. SUSHI. 3 hits.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
PS50234. VWFA. 1 hit.
[Graphical view ]
ProtoNeti Search...

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: September 3, 2014
This is version 129 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

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