Sequence of CO4A_HUMAN

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
mannan-binding lectin-associated serine protease-2
P0C0L4
Homo sapiens
1744
192785
Reaction
Selective cleavage after Arg223 in complement component C2 (-Ser-Leu-Gly-Arg-/-Lys-Ile-Gln-Ile) and after Arg76 in complement component C4 (-Gly-Leu-Gln-Arg-/-Ala-Leu-Glu-Ile)
Sequences with same EC No.
Sequence
show sequence in fasta format
   0 MRLLWGLIWA SSFFTLSLQK PRLLLFSPSV VHLGVPLSVG VQLQDVPRGQ VVKGSVFLRN
  60 PSRNNVPCSP KVDFTLSSER DFALLSLQVP LKDAKSCGLH QLLRGPEVQL VAHSPWLKDS
 120 LSRTTNIQGI NLLFSSRRGH LFLQTDQPIY NPGQRVRYRV FALDQKMRPS TDTITVMVEN
 180 SHGLRVRKKE VYMPSSIFQD DFVIPDISEP GTWKISARFS DGLESNSSTQ FEVKKYVLPN
 240 FEVKITPGKP YILTVPGHLD EMQLDIQARY IYGKPVQGVA YVRFGLLDED GKKTFFRGLE
 300 SQTKLVNGQS HISLSKAEFQ DALEKLNMGI TDLQGLRLYV AAAIIESPGG EMEEAELTSW
 360 YFVSSPFSLD LSKTKRHLVP GAPFLLQALV REMSGSPASG IPVKVSATVS SPGSVPEVQD
 420 IQQNTDGSGQ VSIPIIIPQT ISELQLSVSA GSPHPAIARL TVAAPPSGGP GFLSIERPDS
 480 RPPRVGDTLN LNLRAVGSGA TFSHYYYMIL SRGQIVFMNR EPKRTLTSVS VFVDHHLAPS
 540 FYFVAFYYHG DHPVANSLRV DVQAGACEGK LELSVDGAKQ YRNGESVKLH LETDSLALVA
 600 LGALDTALYA AGSKSHKPLN MGKVFEAMNS YDLGCGPGGG DSALQVFQAA GLAFSDGDQW
 660 TLSRKRLSCP KEKTTRKKRN VNFQKAINEK LGQYASPTAK RCCQDGVTRL PMMRSCEQRA
 720 ARVQQPDCRE PFLSCCQFAE SLRKKSRDKG QAGLQRALEI LQEEDLIDED DIPVRSFFPE
 780 NWLWRVETVD RFQILTLWLP DSLTTWEIHG LSLSKTKGLC VATPVQLRVF REFHLHLRLP
 840 MSVRRFEQLE LRPVLYNYLD KNLTVSVHVS PVEGLCLAGG GGLAQQVLVP AGSARPVAFS
 900 VVPTAAAAVS LKVVARGSFE FPVGDAVSKV LQIEKEGAIH REELVYELNP LDHRGRTLEI
 960 PGNSDPNMIP DGDFNSYVRV TASDPLDTLG SEGALSPGGV ASLLRLPRGC GEQTMIYLAP
1020 TLAASRYLDK TEQWSTLPPE TKDHAVDLIQ KGYMRIQQFR KADGSYAAWL SRDSSTWLTA
1080 FVLKVLSLAQ EQVGGSPEKL QETSNWLLSQ QQADGSFQDP CPVLDRSMQG GLVGNDETVA
1140 LTAFVTIALH HGLAVFQDEG AEPLKQRVEA SISKANSFLG EKASAGLLGA HAAAITAYAL
1200 TLTKAPVDLL GVAHNNLMAM AQETGDNLYW GSVTGSQSNA VSPTPAPRNP SDPMPQAPAL
1260 WIETTAYALL HLLLHEGKAE MADQASAWLT RQGSFQGGFR STQDTVIALD ALSAYWIASH
1320 TTEERGLNVT LSSTGRNGFK SHALQLNNRQ IRGLEEELQF SLGSKINVKV GGNSKGTLKV
1380 LRTYNVLDMK NTTCQDLQIE VTVKGHVEYT MEANEDYEDY EYDELPAKDD PDAPLQPVTP
1440 LQLFEGRRNR RRREAPKVVE EQESRVHYTV CIWRNGKVGL SGMAIADVTL LSGFHALRAD
1500 LEKLTSLSDR YVSHFETEGP HVLLYFDSVP TSRECVGFEA VQEVPVGLVQ PASATLYDYY
1560 NPERRCSVFY GAPSKSRLLA TLCSAEVCQC AEGKCPRQRR ALERGLQDED GYRMKFACYY
1620 PRVEYGFQVK VLREDSRAAF RLFETKITQV LHFTKDVKAA ANQMRNFLVR ASCRLRLEPG
1680 KEYLIMGLDG ATYDLEGHPQ YLLDSNSWIE EMPSERLCRS TRQRAACAQL NDFLQEYGTQ
1740 GCQV
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
258410
Belt K.T.,Carroll M.C.,Porter R.R.
The structural basis of the multiple forms of human complement component C4.
Cell
36
907-914
1984
258411
Yu C.Y.
The complete exon-intron structure of a human complement component C4A gene. DNA sequences, polymorphism, and linkage to the 21-hydroxylase gene.
J. Immunol.
146
1057-1066
1991
258413
Mungall A.J.,Palmer S.A.,Sims S.K.,Edwards C.A.,Ashurst J.L.,Wilming L.,Jones M.C.,Horton R.,Hunt S.E.,Scott C.E.,Gilbert J.G.R.,Clamp M.E.,Bethel G.,Milne S.,Ainscough R.,Almeida J.P.,Ambrose K.D.,Andrews T.D.,Ashwell R.I.S.,Babbage A.K.,Bagguley C.L.,Bailey J.,Banerjee R.,Barker D.J.,Barlow K.F.,Bates K.,Beare D.M.,Beasley H.,Beasley O.,Bird C.P.,Blakey S.E.,Bray-Allen S.,Brook J.,Brown A.J.,Brown J.Y.,Burford D.C.,Burrill W.,Burton J.,Carder C.,Carter N.P.,Chapman J.C.,Clark S.Y.,Clark G.,Clee C.M.,Clegg S.,Cobley V.,Collier R.E.,Collins J.E.,Colman L.K.,Corby N.R.,Coville G.J.,Culley K.M.,Dhami P.,Davies J.,Dunn M.,Earthrowl M.E.,Ellington A.E.,Evans K.A.,Faulkner L.,Francis M.D.,Frankish A.,Frankland J.,French L.,Garner P.,Garnett J.,Ghori M.J.,Gilby L.M.,Gillson C.J.,Glithero R.J.,Grafham D.V.,Grant M.,Gribble S.,Griffiths C.,Griffiths M.N.D.,Hall R.,Halls K.S.,Hammond S.,Harley J.L.,Hart E.A.,Heath P.D.,Heathcott R.,Holmes S.J.,Howden P.J.,Howe K.L.,Howell G.R.,Huckle E.,Humphray S.J.,Humphries M.D.,Hunt A.R.,Johnson C.M.,Joy A.A.,Kay M.,Keenan S.J.,Kimberley A.M.,King A.,Laird G.K.,Langford C.,Lawlor S.,Leongamornlert D.A.,Leversha M.,Lloyd C.R.,Lloyd D.M.,Loveland J.E.,Lovell J.,Martin S.,Mashreghi-Mohammadi M.,Maslen G.L.,Matthews L.,McCann O.T.,McLaren S.J.,McLay K.,McMurray A.,Moore M.J.F.,Mullikin J.C.,Niblett D.,Nickerson T.,Novik K.L.,Oliver K.,Overton-Larty E.K.,Parker A.,Patel R.,Pearce A.V.,Peck A.I.,Phillimore B.J.C.T.,Phillips S.,Plumb R.W.,Porter K.M.,Ramsey Y.,Ranby S.A.,Rice C.M.,Ross M.T.,Searle S.M.,Sehra H.K.,Sheridan E.,Skuce C.D.,Smith S.,Smith M.,Spraggon L.,Squares S.L.,Steward C.A.,Sycamore N.,Tamlyn-Hall G.,Tester J.,Theaker A.J.,Thomas D.W.,Thorpe A.,Tracey A.,Tromans A.,Tubby B.,Wall M.,Wallis J.M.,West A.P.,White S.S.,Whitehead S.L.,Whittaker H.,Wild A.,Willey D.J.,Wilmer T.E.,Wood J.M.,Wray P.W.,Wyatt J.C.,Young L.,Younger R.M.,Bentley D.R.,Coulson A.,Durbin R.M.,Hubbard T.,Sulston J.E.,Dunham I.,Rogers J.,Beck S.
The DNA sequence and analysis of human chromosome 6.
Nature
425
805-811
2003
258414
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
258415
Belt K.T.,Yu C.Y.,Carroll M.C.,Porter R.R.
Polymorphism of human complement component C4.
Immunogenetics
21
173-180
1985
258416
Sargent C.A.,Anderson M.J.,Hsieh S.-L.,Kendall E.,Gomez-Escobar N.,Campbell R.D.
Characterisation of the novel gene G11 lying adjacent to the complement C4A gene in the human major histocompatibility complex.
Hum. Mol. Genet.
3
481-488
1994
258418
Moon K.E.,Gorski J.P.,Hugli T.E.
Complete primary structure of human C4a anaphylatoxin.
J. Biol. Chem.
256
8685-8692
1981
258419
Hessing M.,van 't Veer C.,Hackeng T.M.,Bouma B.N.,Iwanaga S.
Importance of the alpha 3-fragment of complement C4 for the binding with C4b-binding protein.
FEBS Lett.
271
131-136
1990
258420
Campbell R.D.,Gagnon J.,Porter R.R.
Amino acid sequence around the thiol and reactive acyl groups of human complement component C4.
Biochem. J.
199
359-370
1981
258421
Chakravarti D.N.,Campbell R.D.,Porter R.R.
The chemical structure of the C4d fragment of the human complement component C4.
Mol. Immunol.
24
1187-1197
1987
258422
Harrison R.A.,Thomas M.L.,Tack B.F.
Sequence determination of the thiolester site of the fourth component of human complement.
Proc. Natl. Acad. Sci. U.S.A.
78
7388-7392
1981
258423
Martinez-Quiles N.,Paz-Artal E.,Moreno-Pelayo M.A.,Longas J.,Ferre-Lopez S.,Rosal M.,Arnaiz-Villena A.
C4d DNA sequences of two infrequent human allotypes (C4A13 and C4B12) and the presence of signal sequences enhancing recombination.
J. Immunol.
161
3438-3443
1998
258424
Carroll M.C.,Porter R.R.
Cloning of a human complement component C4 gene.
Proc. Natl. Acad. Sci. U.S.A.
80
264-267
1983
258425
Chakravarti D.N.,Campbell R.D.,Gagnon J.
Amino acid sequence of a polymorphic segment from fragment C4d of human complement component C4.
FEBS Lett.
154
387-390
1983
258426
Hortin G.,Sims H.,Strauss A.W.
Identification of the site of sulfation of the fourth component of human complement.
J. Biol. Chem.
261
1786-1793
1986
258427
Whitehead A.S.,Goldberger G.,Woods D.E.,Markham A.F.,Colten H.R.
Use of a cDNA clone for the fourth component of human complement (C4) for analysis of a genetic deficiency of C4 in guinea pig.
Proc. Natl. Acad. Sci. U.S.A.
80
5387-5391
1983
258428
Carroll M.C.,Fathallah D.M.,Bergamaschini L.,Alicot E.M.,Isenman D.E.
Substitution of a single amino acid (aspartic acid for histidine) converts the functional activity of human complement C4B to C4A.
Proc. Natl. Acad. Sci. U.S.A.
87
6868-6872
1990
258429
Barba G.,Rittner C.,Schneider P.M.
Genetic basis of human complement C4A deficiency. Detection of a point mutation leading to nonexpression.
J. Clin. Invest.
91
1681-1686
1993
258430
Dodds A.W.,Ren X.D.,Willis A.C.,Law S.K.
The reaction mechanism of the internal thioester in the human complement component C4.
Nature
379
177-179
1996
258431
Lokki M.L.,Circolo A.,Ahokas P.,Rupert K.L.,Yu C.Y.,Colten H.R.
Deficiency of human complement protein C4 due to identical frameshift mutations in the C4A and C4B genes.
J. Immunol.
162
3687-3693
1999
258432
Blanchong C.A.,Chung E.K.,Rupert K.L.,Yang Y.,Yang Z.,Zhou B.,Moulds J.M.,Yu C.Y.
Genetic, structural and functional diversities of human complement components C4A and C4B and their mouse homologues, Slp and C4.
Int. Immunopharmacol.
1
365-392
2001
258433
Zhang H.,Li X.-J.,Martin D.B.,Aebersold R.
Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry.
Nat. Biotechnol.
21
660-666
2003
258434
Bunkenborg J.,Pilch B.J.,Podtelejnikov A.V.,Wisniewski J.R.
Screening for N-glycosylated proteins by liquid chromatography mass spectrometry.
Proteomics
4
454-465
2004
258435
Yu C.Y.,Belt K.T.,Giles C.M.,Campbell R.D.,Porter R.R.
Structural basis of the polymorphism of human complement components C4A and C4B: gene size, reactivity and antigenicity.
EMBO J.
5
2873-2881
1986
258436
Liu T.,Qian W.-J.,Gritsenko M.A.,Camp D.G. II,Monroe M.E.,Moore R.J.,Smith R.D.
Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry.
J. Proteome Res.
4
2070-2080
2005
258437
Yang Y.,Chung E.K.,Wu Y.L.,Savelli S.L.,Nagaraja H.N.,Zhou B.,Hebert M.,Jones K.N.,Shu Y.,Kitzmiller K.,Blanchong C.A.,McBride K.L.,Higgins G.C.,Rennebohm R.M.,Rice R.R.,Hackshaw K.V.,Roubey R.A.,Grossman J.M.,Tsao B.P.,Birmingham D.J.,Rovin B.H.,Hebert L.A.,Yu C.Y.
Gene copy-number variation and associated polymorphisms of complement component C4 in human systemic lupus erythematosus (SLE): low copy number is a risk factor for and high copy number is a protective factor against SLE susceptibility in European Americans.
Am. J. Hum. Genet.
80
1037-1054
2007
258438
Picariello G.,Ferranti P.,Mamone G.,Roepstorff P.,Addeo F.
Identification of N-linked glycoproteins in human milk by hydrophilic interaction liquid chromatography and mass spectrometry.
Proteomics
8
3833-3847
2008
258439
Chen R.,Jiang X.,Sun D.,Han G.,Wang F.,Ye M.,Wang L.,Zou H.
Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry.
J. Proteome Res.
8
651-661
2009
258440
Jia W.,Lu Z.,Fu Y.,Wang H.P.,Wang L.H.,Chi H.,Yuan Z.F.,Zheng Z.B.,Song L.N.,Han H.H.,Liang Y.M.,Wang J.L.,Cai Y.,Zhang Y.K.,Deng Y.L.,Ying W.T.,He S.M.,Qian X.H.
A strategy for precise and large scale identification of core fucosylated glycoproteins.
Mol. Cell. Proteomics
8
913-923
2009
258441
Nilsson J.,Rueetschi U.,Halim A.,Hesse C.,Carlsohn E.,Brinkmalm G.,Larson G.
Enrichment of glycopeptides for glycan structure and attachment site identification.
Nat. Methods
6
809-811
2009
258442
Halim A.,Ruetschi U.,Larson G.,Nilsson J.
LC-MS/MS characterization of O-glycosylation sites and glycan structures of human cerebrospinal fluid glycoproteins.
J. Proteome Res.
12
573-584
2013
258443
Tagliabracci V.S.,Wiley S.E.,Guo X.,Kinch L.N.,Durrant E.,Wen J.,Xiao J.,Cui J.,Nguyen K.B.,Engel J.L.,Coon J.J.,Grishin N.,Pinna L.A.,Pagliarini D.J.,Dixon J.E.
A single kinase generates the majority of the secreted phosphoproteome.
Cell
161
1619-1632
2015
258444
Sekar A.,Bialas A.R.,de Rivera H.,Davis A.,Hammond T.R.,Kamitaki N.,Tooley K.,Presumey J.,Baum M.,Van Doren V.,Genovese G.,Rose S.A.,Handsaker R.E.,Daly M.J.,Carroll M.C.,Stevens B.,McCarroll S.A.
Schizophrenia risk from complex variation of complement component 4.
Nature
530
177-183
2016
258445
van den Elsen J.M.,Martin A.,Wong V.,Clemenza L.,Rose D.R.,Isenman D.E.
X-ray crystal structure of the C4d fragment of human complement component C4.
J. Mol. Biol.
322
1103-1115
2002
258446
Anderson M.J.,Milner C.M.,Cotton G.H.,Campbell R.D.
The coding sequence of the hemolytically inactive C4A6 allotype of human complement component C4 reveals that a single arginine to tryptophan substitution at beta-chain residue 458 is the likely cause of the defect.
J. Immunol.
148
2795-2802
1992