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P0C0L4

- CO4A_HUMAN

UniProt

P0C0L4 - CO4A_HUMAN

Protein

Complement C4-A

Gene

C4A

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 105 (01 Oct 2014)
      Sequence version 2 (03 Apr 2013)
      Previous versions | rss
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    Functioni

    Non-enzymatic component of C3 and C5 convertases and thus essential for the propagation of the classical complement pathway. Covalently binds to immunoglobulins and immune complexes and enhances the solubilization of immune aggregates and the clearance of IC through CR1 on erythrocytes. C4A isotype is responsible for effective binding to form amide bonds with immune aggregates or protein antigens, while C4B isotype catalyzes the transacylation of the thioester carbonyl group to form ester bonds with carbohydrate antigens.
    Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sitei1125 – 11251Responsible for effective binding to form amide bonds with immune aggregates or protein antigens

    GO - Molecular functioni

    1. complement component C1q binding Source: BHF-UCL
    2. endopeptidase inhibitor activity Source: InterPro

    GO - Biological processi

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

    Keywords - Molecular functioni

    Blood group antigen

    Keywords - Biological processi

    Complement pathway, Immunity, Inflammatory response, Innate immunity

    Enzyme and pathway databases

    ReactomeiREACT_118707. Regulation of Complement cascade.
    REACT_7972. Activation of C3 and C5.
    REACT_8024. Initial triggering of complement.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Complement C4-A
    Alternative name(s):
    Acidic complement C4
    C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2
    Cleaved into the following 6 chains:
    Gene namesi
    Name:C4A
    Synonyms:CO4, CPAMD2
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 6

    Organism-specific databases

    HGNCiHGNC:1323. C4A.

    Subcellular locationi

    GO - Cellular componenti

    1. blood microparticle Source: UniProt
    2. extracellular region Source: Reactome
    3. plasma membrane Source: Reactome

    Keywords - Cellular componenti

    Secreted

    Pathology & Biotechi

    Involvement in diseasei

    Complement component 4A deficiency (C4AD) [MIM:614380]: A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Systemic lupus erythematosus (SLE) [MIM:152700]: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.2 Publications
    Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Interindividual copy-number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE.

    Keywords - Diseasei

    Disease mutation, Systemic lupus erythematosus

    Organism-specific databases

    MIMi120790. phenotype.
    152700. phenotype.
    614374. phenotype.
    614380. phenotype.
    Orphaneti117. Behcet disease.
    169147. Immunodeficiency due to an early component of complement deficiency.
    536. Systemic lupus erythematosus.
    PharmGKBiPA25903.
    PA25904.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Signal peptidei1 – 1919Add
    BLAST
    Chaini20 – 675656Complement C4 beta chainPRO_0000005966Add
    BLAST
    Propeptidei676 – 67941 PublicationPRO_0000005967
    Chaini680 – 1446767Complement C4-A alpha chainPRO_0000005968Add
    BLAST
    Chaini680 – 75677C4a anaphylatoxinPRO_0000005969Add
    BLAST
    Chaini757 – 1446690C4b-APRO_0000005970Add
    BLAST
    Chaini957 – 1336380C4d-APRO_0000042698Add
    BLAST
    Propeptidei1447 – 14537PRO_0000005971
    Chaini1454 – 1744291Complement C4 gamma chainPRO_0000005972Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Glycosylationi226 – 2261N-linked (GlcNAc...)4 Publications
    Disulfide bondi702 ↔ 728By similarity
    Disulfide bondi703 ↔ 735By similarity
    Disulfide bondi716 ↔ 736By similarity
    Glycosylationi862 – 8621N-linked (GlcNAc...)2 Publications
    Cross-linki1010 ↔ 1013Isoglutamyl cysteine thioester (Cys-Gln)
    Glycosylationi1244 – 12441O-linked (GalNAc...)2 Publications
    Glycosylationi1328 – 13281N-linked (GlcNAc...) (complex)5 Publications
    Glycosylationi1391 – 13911N-linked (GlcNAc...)4 Publications
    Modified residuei1417 – 14171Sulfotyrosine1 Publication
    Modified residuei1420 – 14201Sulfotyrosine1 Publication
    Modified residuei1422 – 14221Sulfotyrosine1 Publication
    Disulfide bondi1595 ↔ 1673By similarity
    Disulfide bondi1618 ↔ 1742By similarity

    Post-translational modificationi

    Prior to secretion, the single-chain precursor is enzymatically cleaved to yield non-identical chains alpha, beta and gamma. During activation, the alpha chain is cleaved by C1 into C4a and C4b, and C4b stays linked to the beta and gamma chains. Further degradation of C4b by C1 into the inactive fragments C4c and C4d blocks the generation of C3 convertase. The proteolytic cleavages often are incomplete so that many structural forms can be found in plasma.
    N- and O-glycosylated. O-glycosylated with a core 1 or possibly core 8 glycan.8 Publications

    Keywords - PTMi

    Cleavage on pair of basic residues, Disulfide bond, Glycoprotein, Sulfation, Thioester bond

    Proteomic databases

    PaxDbiP0C0L4.
    PRIDEiP0C0L4.

    PTM databases

    PhosphoSiteiP0C0L4.

    Miscellaneous databases

    PMAP-CutDBB0QZR6.

    Expressioni

    Tissue specificityi

    Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland,and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extra-hepatic sites of expression may be important for the local protection and inflammatory response.1 Publication

    Gene expression databases

    BgeeiP0C0L4.
    GenevestigatoriP0C0L4.

    Organism-specific databases

    HPAiCAB009811.
    CAB032603.
    HPA046356.
    HPA048287.
    HPA050103.

    Interactioni

    Subunit structurei

    Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain.

    Protein-protein interaction databases

    BioGridi107181. 4 interactions.
    IntActiP0C0L4. 11 interactions.
    STRINGi9606.ENSP00000364444.

    Structurei

    Secondary structure

    1
    1744
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Helixi997 – 10015
    Helixi1011 – 103020
    Helixi1041 – 105717
    Helixi1076 – 108914
    Helixi1090 – 10923
    Helixi1097 – 110711
    Helixi1108 – 11103
    Helixi1126 – 11327
    Helixi1137 – 115317
    Turni1158 – 11614
    Helixi1162 – 118524
    Helixi1190 – 120213
    Helixi1207 – 121812
    Helixi1259 – 127517
    Helixi1280 – 129213
    Helixi1302 – 131918

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    1HZFX-ray2.30A957-1323[»]
    4FXGX-ray3.75A/D20-675[»]
    B/E680-1446[»]
    C/F1454-1744[»]
    4FXKX-ray3.60A20-675[»]
    B680-1446[»]
    C1454-1744[»]
    ProteinModelPortaliP0C0L4.
    SMRiP0C0L4. Positions 20-670, 681-1420, 1455-1744.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiP0C0L4.

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini702 – 73635Anaphylatoxin-likePROSITE-ProRule annotationAdd
    BLAST
    Domaini1595 – 1742148NTRPROSITE-ProRule annotationAdd
    BLAST

    Sequence similaritiesi

    Contains 1 anaphylatoxin-like domain.PROSITE-ProRule annotation
    Contains 1 NTR domain.PROSITE-ProRule annotation

    Keywords - Domaini

    Signal

    Phylogenomic databases

    eggNOGiCOG2373.
    HOVERGENiHBG107123.
    InParanoidiP0C0L4.
    KOiK03989.
    OMAiTHQGGFQ.
    PhylomeDBiP0C0L4.
    TreeFamiTF313285.

    Family and domain databases

    Gene3Di1.20.91.20. 1 hit.
    1.50.10.20. 1 hit.
    2.60.40.690. 1 hit.
    InterProiIPR009048. A-macroglobulin_rcpt-bd.
    IPR011626. A2M_comp.
    IPR002890. A2M_N.
    IPR011625. A2M_N_2.
    IPR000020. Anaphylatoxin/fibulin.
    IPR018081. Anaphylatoxin_comp_syst.
    IPR001840. Anaphylatoxn_comp_syst_dom.
    IPR001599. Macroglobln_a2.
    IPR019742. MacrogloblnA2_CS.
    IPR019565. MacrogloblnA2_thiol-ester-bond.
    IPR001134. Netrin_domain.
    IPR018933. Netrin_module_non-TIMP.
    IPR008930. Terpenoid_cyclase/PrenylTrfase.
    IPR008993. TIMP-like_OB-fold.
    [Graphical view]
    PfamiPF00207. A2M. 1 hit.
    PF07678. A2M_comp. 1 hit.
    PF01835. A2M_N. 1 hit.
    PF07703. A2M_N_2. 1 hit.
    PF07677. A2M_recep. 1 hit.
    PF01821. ANATO. 1 hit.
    PF01759. NTR. 1 hit.
    PF10569. Thiol-ester_cl. 1 hit.
    [Graphical view]
    PRINTSiPR00004. ANAPHYLATOXN.
    SMARTiSM00104. ANATO. 1 hit.
    SM00643. C345C. 1 hit.
    [Graphical view]
    SUPFAMiSSF47686. SSF47686. 1 hit.
    SSF48239. SSF48239. 1 hit.
    SSF49410. SSF49410. 1 hit.
    SSF50242. SSF50242. 1 hit.
    PROSITEiPS00477. ALPHA_2_MACROGLOBULIN. 1 hit.
    PS01177. ANAPHYLATOXIN_1. 1 hit.
    PS01178. ANAPHYLATOXIN_2. 1 hit.
    PS50189. NTR. 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 1 (identifier: P0C0L4-1) [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

    MRLLWGLIWA SSFFTLSLQK PRLLLFSPSV VHLGVPLSVG VQLQDVPRGQ     50
    VVKGSVFLRN PSRNNVPCSP KVDFTLSSER DFALLSLQVP LKDAKSCGLH 100
    QLLRGPEVQL VAHSPWLKDS LSRTTNIQGI NLLFSSRRGH LFLQTDQPIY 150
    NPGQRVRYRV FALDQKMRPS TDTITVMVEN SHGLRVRKKE VYMPSSIFQD 200
    DFVIPDISEP GTWKISARFS DGLESNSSTQ FEVKKYVLPN FEVKITPGKP 250
    YILTVPGHLD EMQLDIQARY IYGKPVQGVA YVRFGLLDED GKKTFFRGLE 300
    SQTKLVNGQS HISLSKAEFQ DALEKLNMGI TDLQGLRLYV AAAIIESPGG 350
    EMEEAELTSW YFVSSPFSLD LSKTKRHLVP GAPFLLQALV REMSGSPASG 400
    IPVKVSATVS SPGSVPEVQD IQQNTDGSGQ VSIPIIIPQT ISELQLSVSA 450
    GSPHPAIARL TVAAPPSGGP GFLSIERPDS RPPRVGDTLN LNLRAVGSGA 500
    TFSHYYYMIL SRGQIVFMNR EPKRTLTSVS VFVDHHLAPS FYFVAFYYHG 550
    DHPVANSLRV DVQAGACEGK LELSVDGAKQ YRNGESVKLH LETDSLALVA 600
    LGALDTALYA AGSKSHKPLN MGKVFEAMNS YDLGCGPGGG DSALQVFQAA 650
    GLAFSDGDQW TLSRKRLSCP KEKTTRKKRN VNFQKAINEK LGQYASPTAK 700
    RCCQDGVTRL PMMRSCEQRA ARVQQPDCRE PFLSCCQFAE SLRKKSRDKG 750
    QAGLQRALEI LQEEDLIDED DIPVRSFFPE NWLWRVETVD RFQILTLWLP 800
    DSLTTWEIHG LSLSKTKGLC VATPVQLRVF REFHLHLRLP MSVRRFEQLE 850
    LRPVLYNYLD KNLTVSVHVS PVEGLCLAGG GGLAQQVLVP AGSARPVAFS 900
    VVPTAAAAVS LKVVARGSFE FPVGDAVSKV LQIEKEGAIH REELVYELNP 950
    LDHRGRTLEI PGNSDPNMIP DGDFNSYVRV TASDPLDTLG SEGALSPGGV 1000
    ASLLRLPRGC GEQTMIYLAP TLAASRYLDK TEQWSTLPPE TKDHAVDLIQ 1050
    KGYMRIQQFR KADGSYAAWL SRDSSTWLTA FVLKVLSLAQ EQVGGSPEKL 1100
    QETSNWLLSQ QQADGSFQDP CPVLDRSMQG GLVGNDETVA LTAFVTIALH 1150
    HGLAVFQDEG AEPLKQRVEA SISKANSFLG EKASAGLLGA HAAAITAYAL 1200
    TLTKAPVDLL GVAHNNLMAM AQETGDNLYW GSVTGSQSNA VSPTPAPRNP 1250
    SDPMPQAPAL WIETTAYALL HLLLHEGKAE MADQASAWLT RQGSFQGGFR 1300
    STQDTVIALD ALSAYWIASH TTEERGLNVT LSSTGRNGFK SHALQLNNRQ 1350
    IRGLEEELQF SLGSKINVKV GGNSKGTLKV LRTYNVLDMK NTTCQDLQIE 1400
    VTVKGHVEYT MEANEDYEDY EYDELPAKDD PDAPLQPVTP LQLFEGRRNR 1450
    RRREAPKVVE EQESRVHYTV CIWRNGKVGL SGMAIADVTL LSGFHALRAD 1500
    LEKLTSLSDR YVSHFETEGP HVLLYFDSVP TSRECVGFEA VQEVPVGLVQ 1550
    PASATLYDYY NPERRCSVFY GAPSKSRLLA TLCSAEVCQC AEGKCPRQRR 1600
    ALERGLQDED GYRMKFACYY PRVEYGFQVK VLREDSRAAF RLFETKITQV 1650
    LHFTKDVKAA ANQMRNFLVR ASCRLRLEPG KEYLIMGLDG ATYDLEGHPQ 1700
    YLLDSNSWIE EMPSERLCRS TRQRAACAQL NDFLQEYGTQ GCQV 1744
    Length:1,744
    Mass (Da):192,785
    Last modified:April 3, 2013 - v2
    Checksum:i9396A4CC4DA3602C
    GO
    Isoform 2 (identifier: P0C0L4-2) [UniParc]FASTAAdd to Basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1458-1503: Missing.

    Note: No experimental confirmation available.

    Show »
    Length:1,698
    Mass (Da):187,704
    Checksum:i6169C0C84E28C512
    GO

    Sequence cautioni

    The sequence AAB59537.1 differs from that shown. Reason: During cDNA synthesis, the 5' end has been inverted (PubMed:3838531).
    The sequence BAE06071.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti217 – 2171A → V in AAI71786. (PubMed:15489334)Curated
    Sequence conflicti643 – 6431A → S in AAH63289. (PubMed:15489334)Curated
    Sequence conflicti729 – 7291R → W in AAH63289. (PubMed:15489334)Curated
    Sequence conflicti1013 – 10131Q → E AA sequence (PubMed:6978711)Curated
    Sequence conflicti1013 – 10131Q → E AA sequence (PubMed:3696167)Curated
    Sequence conflicti1013 – 10131Q → E AA sequence (PubMed:6950384)Curated
    Sequence conflicti1109 – 11102SQ → IA AA sequence (PubMed:3696167)Curated
    Sequence conflicti1182 – 11821K → I in AAH63289. (PubMed:15489334)Curated
    Sequence conflicti1245 – 12451P → Q in AAH63289. (PubMed:15489334)Curated
    Sequence conflicti1271 – 12711H → V AA sequence (PubMed:3696167)Curated
    Sequence conflicti1271 – 12711H → V AA sequence (PubMed:6832377)Curated
    Sequence conflicti1300 – 13001R → V AA sequence (PubMed:3696167)Curated
    Sequence conflicti1300 – 13001R → V AA sequence (PubMed:6832377)Curated
    Sequence conflicti1419 – 14213Missing in AAB59537. (PubMed:6546707)Curated
    Sequence conflicti1635 – 16351D → G in AAH12372. (PubMed:15489334)Curated
    Sequence conflicti1637 – 16371R → S in AAI44547. (PubMed:15489334)Curated
    Sequence conflicti1637 – 16371R → S in AAI46850. (PubMed:15489334)Curated
    Sequence conflicti1678 – 16781E → G in AAI71786. (PubMed:15489334)Curated
    Sequence conflicti1704 – 17041D → E in AAH12372. (PubMed:15489334)Curated

    Polymorphismi

    The complement component C4 is the most polymorphic protein of the complement system. It is the product of 2 closely linked and highly homologous genes, C4A and C4B. Once polymorphic variation is discounted, the 2 isotypes differ by only 4 amino acids at positions 1120-1125: PCPVLD for C4A and LSPVIH for C4B. The 2 isotypes bear several antigenic determinants defining Chido/Rodgers blood group system [MIMi:614374]. Rodgers determinants are generally associated with C4A allotypes, and Chido with C4B. Variations at these loci involve not only nucleotide polymorphisms, but also gene number and gene size. Some individuals may lack either C4A, or C4B gene. Partial deficiency of C4A or C4B is the most commonly inherited immune deficiency known in humans with a combined frequency over 31% in the normal Caucasian population (PubMed:11367523). C4A6 allotype is deficient in hemolytic activity. Allotype C4A13 is infrequent.1 Publication

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti141 – 1411L → V.
    Corresponds to variant rs9296005 [ dbSNP | Ensembl ].
    VAR_069154
    Natural varianti347 – 3471S → Y in allotype C4A3a, allotype C4A6. 3 Publications
    Corresponds to variant rs150969927 [ dbSNP | Ensembl ].
    VAR_019778
    Natural varianti418 – 4181V → A in allotype C4A4. 1 Publication
    VAR_069155
    Natural varianti477 – 4771R → W in allotype C4A6. 1 Publication
    VAR_001987
    Natural varianti549 – 5491H → P.1 Publication
    Corresponds to variant rs2229405 [ dbSNP | Ensembl ].
    VAR_069156
    Natural varianti564 – 5641A → D.
    Corresponds to variant rs35277227 [ dbSNP | Ensembl ].
    VAR_069157
    Natural varianti726 – 7261P → L in allotype C4A3a. 1 Publication
    VAR_001988
    Natural varianti727 – 7271D → N.1 Publication
    VAR_019779
    Natural varianti907 – 9071A → T.1 Publication
    Corresponds to variant rs141302872 [ dbSNP | Ensembl ].
    VAR_019780
    Natural varianti1073 – 10731D → G in allotype C4A1, allotype C4A2. 3 Publications
    Corresponds to variant rs147162052 [ dbSNP | Ensembl ].
    VAR_069158
    Natural varianti1176 – 11761N → S in allotype C4A1. 4 Publications
    Corresponds to variant rs17874654 [ dbSNP | Ensembl ].
    VAR_069159
    Natural varianti1201 – 12011T → S in allotype C4A4. 1 Publication
    VAR_001992
    Natural varianti1207 – 12071V → A in allotype C4A1, allotype C4A13. 3 Publications
    Corresponds to variant rs28357075 [ dbSNP | Ensembl ].
    VAR_001993
    Natural varianti1210 – 12101L → R in allotype C4A1, allotype C4A13. 3 Publications
    Corresponds to variant rs28357076 [ dbSNP | Ensembl ].
    VAR_001994
    Natural varianti1286 – 12861S → A in allotype C4A1, allotype C4A3a, allotype C4A6. 6 Publications
    Corresponds to variant rs201016130 [ dbSNP | Ensembl ].
    VAR_001995

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei1458 – 150346Missing in isoform 2. 1 PublicationVSP_046252Add
    BLAST

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    K02403 mRNA. Translation: AAB59537.1. Sequence problems.
    M59815, M59816 Genomic DNA. Translation: AAA51855.1.
    L26261 Genomic DNA. Translation: AAA20121.2.
    AB209989 mRNA. Translation: BAE06071.1. Different initiation.
    AL645922 Genomic DNA. No translation available.
    AL844853 Genomic DNA. No translation available.
    AL929593 Genomic DNA. No translation available.
    CR936924 Genomic DNA. No translation available.
    BC012372 mRNA. Translation: AAH12372.2.
    BC063289 mRNA. Translation: AAH63289.1.
    BC144546 mRNA. Translation: AAI44547.1.
    BC146673 mRNA. Translation: AAI46674.1.
    BC146849 mRNA. Translation: AAI46850.1.
    BC151204 mRNA. Translation: AAI51205.1.
    BC171786 mRNA. Translation: AAI71786.1.
    M14824 Genomic DNA. Translation: AAA52292.1.
    X77491 Genomic DNA. Translation: CAA54627.1.
    AY379925 Genomic DNA. Translation: AAR89152.1.
    AY379926 Genomic DNA. Translation: AAR89153.1.
    AY379927 Genomic DNA. Translation: AAR89154.1.
    AY379928 Genomic DNA. Translation: AAR89155.1.
    AY379929 Genomic DNA. Translation: AAR89156.1.
    AY379930 Genomic DNA. Translation: AAR89157.1.
    AY379931 Genomic DNA. Translation: AAR89158.1.
    AY379932 Genomic DNA. Translation: AAR89159.1.
    AY379933 Genomic DNA. Translation: AAR89160.1.
    AY379934 Genomic DNA. Translation: AAR89161.1.
    AY379935 Genomic DNA. Translation: AAR89162.1.
    AY379960 Genomic DNA. Translation: AAR89164.1.
    AY379962 Genomic DNA. Translation: AAR89166.1.
    AY379963 Genomic DNA. Translation: AAR89167.1.
    AY379964 Genomic DNA. Translation: AAR89168.1.
    AY379965 Genomic DNA. Translation: AAR89169.1.
    AY379966 Genomic DNA. Translation: AAR89170.1.
    U77886 Genomic DNA. Translation: AAK49810.1.
    V00502 mRNA. Translation: CAA23760.1.
    K00830 mRNA. Translation: AAA36229.1.
    CCDSiCCDS47404.1. [P0C0L4-1]
    CCDS59005.1. [P0C0L4-2]
    PIRiB20807.
    I56095. C4HU.
    RefSeqiNP_001002029.3. NM_001002029.3.
    NP_001239133.1. NM_001252204.1. [P0C0L4-2]
    NP_009224.2. NM_007293.2. [P0C0L4-1]
    UniGeneiHs.534847.
    Hs.720022.

    Genome annotation databases

    EnsembliENST00000383325; ENSP00000372815; ENSG00000206340.
    ENST00000421274; ENSP00000388662; ENSG00000227746.
    ENST00000428956; ENSP00000396688; ENSG00000244731. [P0C0L4-1]
    ENST00000498271; ENSP00000420212; ENSG00000244731. [P0C0L4-2]
    GeneIDi720.
    721.
    KEGGihsa:720.
    hsa:721.
    UCSCiuc011doz.2. human.

    Polymorphism databases

    DMDMi476007827.

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    Cross-referencesi

    Web resourcesi

    dbRBC/BGMUT

    Blood group antigen gene mutation database

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    K02403 mRNA. Translation: AAB59537.1 . Sequence problems.
    M59815 , M59816 Genomic DNA. Translation: AAA51855.1 .
    L26261 Genomic DNA. Translation: AAA20121.2 .
    AB209989 mRNA. Translation: BAE06071.1 . Different initiation.
    AL645922 Genomic DNA. No translation available.
    AL844853 Genomic DNA. No translation available.
    AL929593 Genomic DNA. No translation available.
    CR936924 Genomic DNA. No translation available.
    BC012372 mRNA. Translation: AAH12372.2 .
    BC063289 mRNA. Translation: AAH63289.1 .
    BC144546 mRNA. Translation: AAI44547.1 .
    BC146673 mRNA. Translation: AAI46674.1 .
    BC146849 mRNA. Translation: AAI46850.1 .
    BC151204 mRNA. Translation: AAI51205.1 .
    BC171786 mRNA. Translation: AAI71786.1 .
    M14824 Genomic DNA. Translation: AAA52292.1 .
    X77491 Genomic DNA. Translation: CAA54627.1 .
    AY379925 Genomic DNA. Translation: AAR89152.1 .
    AY379926 Genomic DNA. Translation: AAR89153.1 .
    AY379927 Genomic DNA. Translation: AAR89154.1 .
    AY379928 Genomic DNA. Translation: AAR89155.1 .
    AY379929 Genomic DNA. Translation: AAR89156.1 .
    AY379930 Genomic DNA. Translation: AAR89157.1 .
    AY379931 Genomic DNA. Translation: AAR89158.1 .
    AY379932 Genomic DNA. Translation: AAR89159.1 .
    AY379933 Genomic DNA. Translation: AAR89160.1 .
    AY379934 Genomic DNA. Translation: AAR89161.1 .
    AY379935 Genomic DNA. Translation: AAR89162.1 .
    AY379960 Genomic DNA. Translation: AAR89164.1 .
    AY379962 Genomic DNA. Translation: AAR89166.1 .
    AY379963 Genomic DNA. Translation: AAR89167.1 .
    AY379964 Genomic DNA. Translation: AAR89168.1 .
    AY379965 Genomic DNA. Translation: AAR89169.1 .
    AY379966 Genomic DNA. Translation: AAR89170.1 .
    U77886 Genomic DNA. Translation: AAK49810.1 .
    V00502 mRNA. Translation: CAA23760.1 .
    K00830 mRNA. Translation: AAA36229.1 .
    CCDSi CCDS47404.1. [P0C0L4-1 ]
    CCDS59005.1. [P0C0L4-2 ]
    PIRi B20807.
    I56095. C4HU.
    RefSeqi NP_001002029.3. NM_001002029.3.
    NP_001239133.1. NM_001252204.1. [P0C0L4-2 ]
    NP_009224.2. NM_007293.2. [P0C0L4-1 ]
    UniGenei Hs.534847.
    Hs.720022.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    1HZF X-ray 2.30 A 957-1323 [» ]
    4FXG X-ray 3.75 A/D 20-675 [» ]
    B/E 680-1446 [» ]
    C/F 1454-1744 [» ]
    4FXK X-ray 3.60 A 20-675 [» ]
    B 680-1446 [» ]
    C 1454-1744 [» ]
    ProteinModelPortali P0C0L4.
    SMRi P0C0L4. Positions 20-670, 681-1420, 1455-1744.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 107181. 4 interactions.
    IntActi P0C0L4. 11 interactions.
    STRINGi 9606.ENSP00000364444.

    PTM databases

    PhosphoSitei P0C0L4.

    Polymorphism databases

    DMDMi 476007827.

    Proteomic databases

    PaxDbi P0C0L4.
    PRIDEi P0C0L4.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000383325 ; ENSP00000372815 ; ENSG00000206340 .
    ENST00000421274 ; ENSP00000388662 ; ENSG00000227746 .
    ENST00000428956 ; ENSP00000396688 ; ENSG00000244731 . [P0C0L4-1 ]
    ENST00000498271 ; ENSP00000420212 ; ENSG00000244731 . [P0C0L4-2 ]
    GeneIDi 720.
    721.
    KEGGi hsa:720.
    hsa:721.
    UCSCi uc011doz.2. human.

    Organism-specific databases

    CTDi 720.
    721.
    GeneCardsi GC06P031954.
    GC06Pm32059.
    GC06Pn31940.
    HGNCi HGNC:1323. C4A.
    HPAi CAB009811.
    CAB032603.
    HPA046356.
    HPA048287.
    HPA050103.
    MIMi 120790. phenotype.
    120810. gene.
    152700. phenotype.
    614374. phenotype.
    614380. phenotype.
    neXtProti NX_P0C0L4.
    Orphaneti 117. Behcet disease.
    169147. Immunodeficiency due to an early component of complement deficiency.
    536. Systemic lupus erythematosus.
    PharmGKBi PA25903.
    PA25904.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG2373.
    HOVERGENi HBG107123.
    InParanoidi P0C0L4.
    KOi K03989.
    OMAi THQGGFQ.
    PhylomeDBi P0C0L4.
    TreeFami TF313285.

    Enzyme and pathway databases

    Reactomei REACT_118707. Regulation of Complement cascade.
    REACT_7972. Activation of C3 and C5.
    REACT_8024. Initial triggering of complement.

    Miscellaneous databases

    EvolutionaryTracei P0C0L4.
    GeneWikii C4A.
    NextBioi 13630656.
    PMAP-CutDB B0QZR6.
    PROi P0C0L4.
    SOURCEi Search...

    Gene expression databases

    Bgeei P0C0L4.
    Genevestigatori P0C0L4.

    Family and domain databases

    Gene3Di 1.20.91.20. 1 hit.
    1.50.10.20. 1 hit.
    2.60.40.690. 1 hit.
    InterProi IPR009048. A-macroglobulin_rcpt-bd.
    IPR011626. A2M_comp.
    IPR002890. A2M_N.
    IPR011625. A2M_N_2.
    IPR000020. Anaphylatoxin/fibulin.
    IPR018081. Anaphylatoxin_comp_syst.
    IPR001840. Anaphylatoxn_comp_syst_dom.
    IPR001599. Macroglobln_a2.
    IPR019742. MacrogloblnA2_CS.
    IPR019565. MacrogloblnA2_thiol-ester-bond.
    IPR001134. Netrin_domain.
    IPR018933. Netrin_module_non-TIMP.
    IPR008930. Terpenoid_cyclase/PrenylTrfase.
    IPR008993. TIMP-like_OB-fold.
    [Graphical view ]
    Pfami PF00207. A2M. 1 hit.
    PF07678. A2M_comp. 1 hit.
    PF01835. A2M_N. 1 hit.
    PF07703. A2M_N_2. 1 hit.
    PF07677. A2M_recep. 1 hit.
    PF01821. ANATO. 1 hit.
    PF01759. NTR. 1 hit.
    PF10569. Thiol-ester_cl. 1 hit.
    [Graphical view ]
    PRINTSi PR00004. ANAPHYLATOXN.
    SMARTi SM00104. ANATO. 1 hit.
    SM00643. C345C. 1 hit.
    [Graphical view ]
    SUPFAMi SSF47686. SSF47686. 1 hit.
    SSF48239. SSF48239. 1 hit.
    SSF49410. SSF49410. 1 hit.
    SSF50242. SSF50242. 1 hit.
    PROSITEi PS00477. ALPHA_2_MACROGLOBULIN. 1 hit.
    PS01177. ANAPHYLATOXIN_1. 1 hit.
    PS01178. ANAPHYLATOXIN_2. 1 hit.
    PS50189. NTR. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "The structural basis of the multiple forms of human complement component C4."
      Belt K.T., Carroll M.C., Porter R.R.
      Cell 36:907-914(1984) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), VARIANTS ALA-418 AND SER-1201.
      Tissue: Liver.
    2. "The complete exon-intron structure of a human complement component C4A gene. DNA sequences, polymorphism, and linkage to the 21-hydroxylase gene."
      Yu C.Y.
      J. Immunol. 146:1057-1066(1991) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS TYR-347; LEU-726 AND ALA-1286.
    3. "Preparation of a set of expression-ready clones of mammalian long cDNAs encoding large proteins by the ORF trap cloning method."
      Nakajima D., Saito K., Yamakawa H., Kikuno R.F., Nakayama M., Ohara R., Okazaki N., Koga H., Nagase T., Ohara O.
      Submitted (MAR-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), VARIANTS SER-1176 AND ALA-1286.
      Tissue: Brain.
    4. "The DNA sequence and analysis of human chromosome 6."
      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.
      Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANTS TYR-347; SER-1176 AND ALA-1286.
    5. "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] (ISOFORMS 1 AND 2), VARIANTS TYR-347; GLY-1073 AND ALA-1286.
      Tissue: Brain.
    6. "Polymorphism of human complement component C4."
      Belt K.T., Yu C.Y., Carroll M.C., Porter R.R.
      Immunogenetics 21:173-180(1985) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-22 AND 1056-1225.
    7. "Characterisation of the novel gene G11 lying adjacent to the complement C4A gene in the human major histocompatibility complex."
      Sargent C.A., Anderson M.J., Hsieh S.-L., Kendall E., Gomez-Escobar N., Campbell R.D.
      Hum. Mol. Genet. 3:481-488(1994) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-21, VARIANT ASN-727.
    8. "Molecular genetics of complement C4: implications for MHC evolution and disease susceptibility gene mapping."
      Sayer D., Puschendorf M., Wetherall J.
      Submitted (SEP-2003) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 448-570 AND 692-1225, VARIANTS TRP-477; PRO-549; THR-907; GLY-1073; SER-1176; ALA-1207 AND ARG-1210.
    9. "Complete primary structure of human C4a anaphylatoxin."
      Moon K.E., Gorski J.P., Hugli T.E.
      J. Biol. Chem. 256:8685-8692(1981) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 680-756.
    10. "Importance of the alpha 3-fragment of complement C4 for the binding with C4b-binding protein."
      Hessing M., van 't Veer C., Hackeng T.M., Bouma B.N., Iwanaga S.
      FEBS Lett. 271:131-136(1990) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 757-771 AND 980-990.
    11. "Amino acid sequence around the thiol and reactive acyl groups of human complement component C4."
      Campbell R.D., Gagnon J., Porter R.R.
      Biochem. J. 199:359-370(1981) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 957-1044.
    12. "The chemical structure of the C4d fragment of the human complement component C4."
      Chakravarti D.N., Campbell R.D., Porter R.R.
      Mol. Immunol. 24:1187-1197(1987) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 957-1336, VARIANTS GLY-1073; SER-1176; ALA-1207; ARG-1210 AND ALA-1286.
    13. "Sequence determination of the thiolester site of the fourth component of human complement."
      Harrison R.A., Thomas M.L., Tack B.F.
      Proc. Natl. Acad. Sci. U.S.A. 78:7388-7392(1981) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 990-1037.
    14. "C4d DNA sequences of two infrequent human allotypes (C4A13 and C4B12) and the presence of signal sequences enhancing recombination."
      Martinez-Quiles N., Paz-Artal E., Moreno-Pelayo M.A., Longas J., Ferre-Lopez S., Rosal M., Arnaiz-Villena A.
      J. Immunol. 161:3438-3443(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1055-1225 (ALLOTYPE C4A13).
    15. Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1195-1294.
    16. "Amino acid sequence of a polymorphic segment from fragment C4d of human complement component C4."
      Chakravarti D.N., Campbell R.D., Gagnon J.
      FEBS Lett. 154:387-390(1983) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 1199-1304, VARIANTS ALA-1207; ARG-1210 AND ALA-1286.
    17. "Identification of the site of sulfation of the fourth component of human complement."
      Hortin G., Sims H., Strauss A.W.
      J. Biol. Chem. 261:1786-1793(1986) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 1405-1431, SULFATION AT TYR-1417; TYR-1420 AND TYR-1422.
    18. "Use of a cDNA clone for the fourth component of human complement (C4) for analysis of a genetic deficiency of C4 in guinea pig."
      Whitehead A.S., Goldberger G., Woods D.E., Markham A.F., Colten H.R.
      Proc. Natl. Acad. Sci. U.S.A. 80:5387-5391(1983) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1448-1474.
    19. "Substitution of a single amino acid (aspartic acid for histidine) converts the functional activity of human complement C4B to C4A."
      Carroll M.C., Fathallah D.M., Bergamaschini L., Alicot E.M., Isenman D.E.
      Proc. Natl. Acad. Sci. U.S.A. 87:6868-6872(1990) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, INVOLVEMENT OF ASP-1125 IN IMMUNOGLOBULIN-BINDING AND HEMOLYSIS.
    20. "Genetic basis of human complement C4A deficiency. Detection of a point mutation leading to nonexpression."
      Barba G., Rittner C., Schneider P.M.
      J. Clin. Invest. 91:1681-1686(1993) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN C4AD.
    21. "The reaction mechanism of the internal thioester in the human complement component C4."
      Dodds A.W., Ren X.D., Willis A.C., Law S.K.
      Nature 379:177-179(1996) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION.
    22. "Deficiency of human complement protein C4 due to identical frameshift mutations in the C4A and C4B genes."
      Lokki M.L., Circolo A., Ahokas P., Rupert K.L., Yu C.Y., Colten H.R.
      J. Immunol. 162:3687-3693(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN SLE.
    23. "Genetic, structural and functional diversities of human complement components C4A and C4B and their mouse homologues, Slp and C4."
      Blanchong C.A., Chung E.K., Rupert K.L., Yang Y., Yang Z., Zhou B., Moulds J.M., Yu C.Y.
      Int. Immunopharmacol. 1:365-392(2001) [PubMed] [Europe PMC] [Abstract]
      Cited for: REVIEW, DESCRIPTION OF ALLOTYPES, TISSUE SPECIFICITY.
    24. "Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."
      Zhang H., Li X.-J., Martin D.B., Aebersold R.
      Nat. Biotechnol. 21:660-666(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION AT ASN-226.
    25. "Screening for N-glycosylated proteins by liquid chromatography mass spectrometry."
      Bunkenborg J., Pilch B.J., Podtelejnikov A.V., Wisniewski J.R.
      Proteomics 4:454-465(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-1391.
      Tissue: Plasma.
    26. "Structural basis of the polymorphism of human complement components C4A and C4B: gene size, reactivity and antigenicity."
      Yu C.Y., Belt K.T., Giles C.M., Campbell R.D., Porter R.R.
      EMBO J. 5:2873-2881(1986) [PubMed] [Europe PMC] [Abstract]
      Cited for: STRUCTURAL BASIS OF POLYMORPHISM.
    27. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
      Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E., Moore R.J., Smith R.D.
      J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-862; ASN-1328 AND ASN-1391.
      Tissue: Plasma.
    28. "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."
      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.
      Am. J. Hum. Genet. 80:1037-1054(2007) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN SLE.
    29. "Identification of N-linked glycoproteins in human milk by hydrophilic interaction liquid chromatography and mass spectrometry."
      Picariello G., Ferranti P., Mamone G., Roepstorff P., Addeo F.
      Proteomics 8:3833-3847(2008) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-226 AND ASN-1328.
      Tissue: Milk.
    30. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
      Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.
      J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-226; ASN-1328 AND ASN-1391.
      Tissue: Liver.
    31. Cited for: GLYCOSYLATION AT ASN-1328.
    32. "Enrichment of glycopeptides for glycan structure and attachment site identification."
      Nilsson J., Rueetschi U., Halim A., Hesse C., Carlsohn E., Brinkmalm G., Larson G.
      Nat. Methods 6:809-811(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS], STRUCTURE OF CARBOHYDRATES.
      Tissue: Cerebrospinal fluid.
    33. "LC-MS/MS characterization of O-glycosylation sites and glycan structures of human cerebrospinal fluid glycoproteins."
      Halim A., Ruetschi U., Larson G., Nilsson J.
      J. Proteome Res. 12:573-584(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION AT THR-1244, IDENTIFICATION BY MASS SPECTROMETRY.
    34. "X-ray crystal structure of the C4d fragment of human complement component C4."
      van den Elsen J.M., Martin A., Wong V., Clemenza L., Rose D.R., Isenman D.E.
      J. Mol. Biol. 322:1103-1115(2002) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (2.3 ANGSTROMS) OF 957-1323.
    35. "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."
      Anderson M.J., Milner C.M., Cotton G.H., Campbell R.D.
      J. Immunol. 148:2795-2802(1992) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT ALA-1286 (ALLOTYPE C4A6).

    Entry informationi

    Entry nameiCO4A_HUMAN
    AccessioniPrimary (citable) accession number: P0C0L4
    Secondary accession number(s): A6H8M8
    , A6NHJ5, A7E2V2, B0QZR6, B0V2C8, B2RUT6, B7ZVZ6, P01028, P78445, Q13160, Q13906, Q14033, Q14835, Q4LE82, Q5JNX2, Q5JQM8, Q6P4R1, Q6U2E5, Q6U2E8, Q6U2F0, Q6U2F3, Q6U2F4, Q6U2F6, Q6U2F8, Q6U2G0, Q96EG2, Q96SA8, Q9NPK5, Q9UIP5
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: July 21, 1986
    Last sequence update: April 3, 2013
    Last modified: October 1, 2014
    This is version 105 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

    Keywords - Technical termi

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

    Documents

    1. Blood group antigen proteins
      Nomenclature of blood group antigens and list of entries
    2. Human chromosome 6
      Human chromosome 6: entries, gene names and cross-references to MIM
    3. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    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. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3