P0C0L4 (CO4A_HUMAN) Reviewed, UniProtKB/Swiss-Prot
Last modified
January 25, 2012.
Version 78.
History...
Names·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order
Names·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize orderNames and origin
| Protein names | 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 names |
| ||||
| Organism | Homo sapiens (Human) | ||||
| Taxonomic identifier | 9606 [NCBI] | ||||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Protein attributes
| Sequence length | 1744 AA. |
| Sequence status | Complete. |
| Sequence processing | The displayed sequence is further processed into a mature form. |
| Protein existence | Evidence at protein level |
General annotation (Comments)
| Function | C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway. 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. |
| Subunit structure | Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain. |
| Subcellular location | |
| Post-translational modification | Prior to secretion, the single-chain precursor is enzymatically cleaved to yield the 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. N- and O-glycosylated. O-glycosylated with a core 1 or possibly core 8 glycan. Ref.15 Ref.16 Ref.18 Ref.20 Ref.21 Ref.22 |
| Polymorphism | Human complement component C4 is polymorphic at two loci, C4A and C4B. 13 alleles of C4A and 22 alleles of C4B have been detected. The allele shown here is C4A4. The C4A alleles carry the Rodgers (Rg) while the C4B alleles carry the Chido (Ch) blood group antigens. The C4A6 allotype is totally deficient in hemolytic activity. |
| Involvement in disease | Defects in C4A are the cause of complement component 4A deficiency (C4AD) [MIM:120810]. A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis. Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE) [MIM:152700]. A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Note=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. Ref.19 |
| Miscellaneous | C4A allotypes react more rapidly with the amino group of peptide antigens while C4B allotypes react more rapidly with the hydroxyl group of carbohydrate antigens. |
| Sequence similarities | Contains 1 anaphylatoxin-like domain. Contains 1 NTR domain. |
Ontologies
| Keywords | |
|---|---|
| Biological process | Complement pathway Immunity Inflammatory response Innate immunity |
| Cellular component | Secreted |
| Coding sequence diversity | Polymorphism |
| Disease | Disease mutation Systemic lupus erythematosus |
| Domain | Signal |
| Molecular function | Blood group antigen |
| PTM | Cleavage on pair of basic residues Disulfide bond Glycoprotein Sulfation Thioester bond |
| Technical term | 3D-structure Complete proteome Direct protein sequencing Reference proteome |
| Gene Ontology (GO) | |
| Biological process | complement activation, classical pathway Inferred from electronic annotation. Source: UniProtKB-KW inflammatory responseInferred from electronic annotation. Source: UniProtKB-KW innate immune responseTraceable author statement. Source: Reactome |
| Cellular component | extracellular space Inferred from electronic annotation. Source: InterPro |
| Molecular function | endopeptidase inhibitor activity Inferred from electronic annotation. Source: InterPro |
| Complete GO annotation... | |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | |||||||||||||||||||||||||||||||||
Molecule processing | ||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Signal peptide | 1 – 19 | 19 | ||||||||||||||||||||||||||||||||||||
| Chain | 20 – 675 | 656 | Complement C4 beta chain | PRO_0000005966 | ||||||||||||||||||||||||||||||||||
| Propeptide | 676 – 679 | 4 | PRO_0000005967 | |||||||||||||||||||||||||||||||||||
| Chain | 680 – 1446 | 767 | Complement C4-A alpha chain | PRO_0000005968 | ||||||||||||||||||||||||||||||||||
| Chain | 680 – 756 | 77 | C4a anaphylatoxin | PRO_0000005969 | ||||||||||||||||||||||||||||||||||
| Chain | 757 – 1446 | 690 | C4b-A | PRO_0000005970 | ||||||||||||||||||||||||||||||||||
| Chain | 957 – 1336 | 380 | C4d-A | PRO_0000042698 | ||||||||||||||||||||||||||||||||||
| Propeptide | 1447 – 1453 | 7 | PRO_0000005971 | |||||||||||||||||||||||||||||||||||
| Chain | 1454 – 1744 | 291 | Complement C4 gamma chain | PRO_0000005972 | ||||||||||||||||||||||||||||||||||
Regions | ||||||||||||||||||||||||||||||||||||||
| Domain | 702 – 736 | 35 | Anaphylatoxin-like | |||||||||||||||||||||||||||||||||||
| Domain | 1595 – 1742 | 148 | NTR | |||||||||||||||||||||||||||||||||||
| Region | 1232 – 1244 | 13 | O-glycosylated at one site | |||||||||||||||||||||||||||||||||||
Amino acid modifications | ||||||||||||||||||||||||||||||||||||||
| Modified residue | 1417 | 1 | Sulfotyrosine Ref.13 | |||||||||||||||||||||||||||||||||||
| Modified residue | 1420 | 1 | Sulfotyrosine Ref.13 | |||||||||||||||||||||||||||||||||||
| Modified residue | 1422 | 1 | Sulfotyrosine Ref.13 | |||||||||||||||||||||||||||||||||||
| Glycosylation | 226 | 1 | N-linked (GlcNAc...) Ref.15 Ref.20 Ref.21 | |||||||||||||||||||||||||||||||||||
| Glycosylation | 862 | 1 | N-linked (GlcNAc...) Ref.18 | |||||||||||||||||||||||||||||||||||
| Glycosylation | 1328 | 1 | N-linked (GlcNAc...) Ref.18 Ref.20 Ref.21 | |||||||||||||||||||||||||||||||||||
| Glycosylation | 1391 | 1 | N-linked (GlcNAc...) Ref.16 Ref.18 Ref.21 | |||||||||||||||||||||||||||||||||||
| Disulfide bond | 702 ↔ 728 | By similarity | ||||||||||||||||||||||||||||||||||||
| Disulfide bond | 703 ↔ 735 | By similarity | ||||||||||||||||||||||||||||||||||||
| Disulfide bond | 716 ↔ 736 | By similarity | ||||||||||||||||||||||||||||||||||||
| Disulfide bond | 1595 ↔ 1673 | By similarity | ||||||||||||||||||||||||||||||||||||
| Disulfide bond | 1618 ↔ 1742 | By similarity | ||||||||||||||||||||||||||||||||||||
| Cross-link | 1010 ↔ 1013 | Isoglutamyl cysteine thioester (Cys-Gln) | ||||||||||||||||||||||||||||||||||||
Natural variations | ||||||||||||||||||||||||||||||||||||||
| Natural variant | 347 | 1 | S → Y. Ref.2 Corresponds to variant rs392610 [ dbSNP | Ensembl ]. | VAR_019778 | ||||||||||||||||||||||||||||||||||
| Natural variant | 477 | 1 | R → W in allotype C4A6. | VAR_001987 | ||||||||||||||||||||||||||||||||||
| Natural variant | 726 | 1 | P → L in allotype C4A3. Ref.2 Corresponds to variant rs2229408 [ dbSNP | Ensembl ]. | VAR_001988 | ||||||||||||||||||||||||||||||||||
| Natural variant | 727 | 1 | D → N. Ref.5 | VAR_019779 | ||||||||||||||||||||||||||||||||||
| Natural variant | 907 | 1 | A → T. Corresponds to variant rs429329 [ dbSNP | Ensembl ]. | VAR_019780 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1073 | 1 | D → G in allotype C4A1. Ref.9 Corresponds to variant rs2258218 [ dbSNP | Ensembl ]. | VAR_001989 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1176 | 1 | N → S in allotype C4A1. Ref.9 Corresponds to variant rs2746414 [ dbSNP | Ensembl ]. | VAR_001991 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1201 | 1 | S → T in allotype C4A6, allotype C4A3 and allotype C4A1. Ref.3 Ref.9 Ref.11 Ref.12 | VAR_001992 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1207 | 1 | V → A in allotype C4A1. Ref.9 Ref.12 Corresponds to variant rs2229403 [ dbSNP | Ensembl ]. | VAR_001993 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1210 | 1 | L → R in allotype C4A1. Ref.9 Ref.12 Corresponds to variant rs2229409 [ dbSNP | Ensembl ]. | VAR_001994 | ||||||||||||||||||||||||||||||||||
| Natural variant | 1286 | 1 | S → A in allotype C4A6, allotype C4A1, allotype C4A3. Ref.9 Ref.12 Ref.24 Corresponds to variant rs9501603 [ dbSNP | Ensembl ]. | VAR_001995 | ||||||||||||||||||||||||||||||||||
Experimental info | ||||||||||||||||||||||||||||||||||||||
| Sequence conflict | 1 – 12 | 12 | MRLLW…IWASS → TRSAPRAASWLEDPREVRSV CLSAT in AAA52292. Ref.1 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 418 | 1 | V → A in AAB59537. Ref.1 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1013 | 1 | Q → E AA sequence Ref.8 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1013 | 1 | Q → E AA sequence Ref.9 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1013 | 1 | Q → E AA sequence Ref.10 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1109 – 1110 | 2 | SQ → IA AA sequence Ref.9 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1271 | 1 | H → V AA sequence Ref.9 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1271 | 1 | H → V AA sequence Ref.12 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1300 | 1 | R → V AA sequence Ref.9 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1300 | 1 | R → V AA sequence Ref.12 | |||||||||||||||||||||||||||||||||||
| Sequence conflict | 1418 – 1420 | 3 | Missing in AAB59537. Ref.1 | |||||||||||||||||||||||||||||||||||
Secondary structure | ||||||||||||||||||||||||||||||||||||||
Helix Strand Turn | ||||||||||||||||||||||||||||||||||||||
| Helix | 997 – 1001 | 5 | ||||||||||||||||||||||||||||||||||||
| Helix | 1011 – 1030 | 20 | ||||||||||||||||||||||||||||||||||||
| Helix | 1041 – 1057 | 17 | ||||||||||||||||||||||||||||||||||||
| Helix | 1076 – 1089 | 14 | ||||||||||||||||||||||||||||||||||||
| Helix | 1090 – 1092 | 3 | ||||||||||||||||||||||||||||||||||||
| Helix | 1097 – 1107 | 11 | ||||||||||||||||||||||||||||||||||||
| Helix | 1108 – 1110 | 3 | ||||||||||||||||||||||||||||||||||||
| Helix | 1126 – 1132 | 7 | ||||||||||||||||||||||||||||||||||||
| Helix | 1137 – 1153 | 17 | ||||||||||||||||||||||||||||||||||||
| Turn | 1158 – 1161 | 4 | ||||||||||||||||||||||||||||||||||||
| Helix | 1162 – 1185 | 24 | ||||||||||||||||||||||||||||||||||||
| Helix | 1190 – 1202 | 13 | ||||||||||||||||||||||||||||||||||||
| Helix | 1207 – 1218 | 12 | ||||||||||||||||||||||||||||||||||||
| Helix | 1259 – 1275 | 17 | ||||||||||||||||||||||||||||||||||||
| Helix | 1280 – 1292 | 13 | ||||||||||||||||||||||||||||||||||||
| Helix | 1302 – 1319 | 18 | ||||||||||||||||||||||||||||||||||||
Sequences
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References
| « Hide 'large scale' references | |
| [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: 6546707] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. 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: 1988494] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS TYR-347 AND LEU-726 (ALLOTYPE C4A3). |
| [3] | "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. Beck S.Nature 425:805-811(2003) [PubMed: 14574404] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA], VARIANT THR-1201. |
| [4] | "Polymorphism of human complement component C4." Belt K.T., Yu C.Y., Carroll M.C., Porter R.R. Immunogenetics 21:173-180(1985) [PubMed: 3838531] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-22 AND 1056-1225. |
| [5] | "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: 8012361] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-21, VARIANT ASN-727. |
| [6] | "Complete primary structure of human C4a anaphylatoxin." Moon K.E., Gorski J.P., Hugli T.E. J. Biol. Chem. 256:8685-8692(1981) [PubMed: 6167582] [Abstract] Cited for: PROTEIN SEQUENCE OF 680-756. |
| [7] | "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: 1699796] [Abstract] Cited for: PROTEIN SEQUENCE OF 757-771 AND 980-990. |
| [8] | "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: 6978711] [Abstract] Cited for: PROTEIN SEQUENCE OF 957-1044. |
| [9] | "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: 3696167] [Abstract] Cited for: PROTEIN SEQUENCE OF 957-1336, VARIANTS GLY-1073; SER-1176; THR-1201; ALA-1207; ARG-1210 AND ALA-1286. |
| [10] | "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: 6950384] [Abstract] Cited for: PROTEIN SEQUENCE OF 990-1037. |
| [11] | "Cloning of a human complement component C4 gene." Carroll M.C., Porter R.R. Proc. Natl. Acad. Sci. U.S.A. 80:264-267(1983) [PubMed: 6572000] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1195-1294, VARIANT THR-1201. |
| [12] | "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: 6832377] [Abstract] Cited for: PROTEIN SEQUENCE OF 1199-1304, VARIANTS THR-1201; ALA-1207; ARG-1210 AND ALA-1286. |
| [13] | "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: 3944109] [Abstract] Cited for: PROTEIN SEQUENCE OF 1405-1431, SULFATION AT TYR-1417; TYR-1420 AND TYR-1422. |
| [14] | "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: 6577433] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1448-1474. |
| [15] | "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: 12754519] [Abstract] Cited for: GLYCOSYLATION AT ASN-226. |
| [16] | "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: 14760718] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-1391, MASS SPECTROMETRY. Tissue: Plasma. |
| [17] | "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: 2431902] [Abstract] Cited for: STRUCTURAL BASIS OF POLYMORPHISM. |
| [18] | "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: 16335952] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-862; ASN-1328 AND ASN-1391, MASS SPECTROMETRY. Tissue: Plasma. |
| [19] | "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. Yu C.Y.Am. J. Hum. Genet. 80:1037-1054(2007) [PubMed: 17503323] [Abstract] Cited for: INVOLVEMENT IN SLE. |
| [20] | "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: 18780401] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-226 AND ASN-1328, MASS SPECTROMETRY. Tissue: Milk. |
| [21] | "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: 19159218] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-226; ASN-1328 AND ASN-1391, MASS SPECTROMETRY. Tissue: Liver. |
| [22] | "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: 19838169] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS], STRUCTURE OF CARBOHYDRATES, MASS SPECTROMETRY. Tissue: Cerebrospinal fluid. |
| [23] | "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: 12367531] [Abstract] Cited for: X-RAY CRYSTALLOGRAPHY (2.3 ANGSTROMS) OF 957-1323. |
| [24] | "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: 1573268] [Abstract] Cited for: VARIANT ALA-1286 (ALLOTYPE C4A6). |
| + | Additional computationally mapped references. |
Web resources
| dbRBC/BGMUT Blood group antigen gene mutation database |
Cross-references
Sequence databases | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| EMBL GenBank DDBJ | K02403 mRNA. Translation: AAB59537.1. M59815, M59816 Genomic DNA. Translation: AAA51855.1. L26261 Genomic DNA. Translation: AAA20121.2. AL645922 Genomic DNA. Translation: CAQ09284.1. M14824 Genomic DNA. Translation: AAA52292.1. X77491 Genomic DNA. Translation: CAA54627.1. K00830 mRNA. Translation: AAA36229.1. V00502 mRNA. Translation: CAA23760.1. | ||||||||||||
| IPI | IPI00032258. | ||||||||||||
| PIR | B20807. C4HU. I56095. | ||||||||||||
| RefSeq | NP_009224.2. NM_007293.2. | ||||||||||||
| UniGene | Hs.534847. Hs.720022. | ||||||||||||
3D structure databases | |||||||||||||
| PDBe RCSB PDB PDBj |
| ||||||||||||
| ProteinModelPortal | P0C0L4. | ||||||||||||
| SMR | P0C0L4. Positions 138-239, 683-745, 764-984, 996-1321, 1324-1743. | ||||||||||||
| ModBase | Search... | ||||||||||||
Protein-protein interaction databases | |||||||||||||
| IntAct | P0C0L4. 9 interactions. | ||||||||||||
| STRING | P0C0L4. | ||||||||||||
PTM databases | |||||||||||||
| PhosphoSite | P0C0L4. | ||||||||||||
Polymorphism databases | |||||||||||||
| DMDM | 81175238. | ||||||||||||
Proteomic databases | |||||||||||||
| PRIDE | P0C0L4. | ||||||||||||
Protocols and materials databases | |||||||||||||
| StructuralBiologyKnowledgebase | Search... | ||||||||||||
Genome annotation databases | |||||||||||||
| Ensembl | ENST00000428956; ENSP00000396688; ENSG00000244731. | ||||||||||||
| GeneID | 720. | ||||||||||||
| KEGG | hsa:720. | ||||||||||||
Organism-specific databases | |||||||||||||
| CTD | 720. | ||||||||||||
| GeneCards | GC06P031954. | ||||||||||||
| HGNC | HGNC:1323. C4A. | ||||||||||||
| HPA | CAB009811. CAB032603. | ||||||||||||
| MIM | 120790. phenotype. 120810. gene+phenotype. 152700. phenotype. | ||||||||||||
| neXtProt | NX_P0C0L4. | ||||||||||||
| Orphanet | 169147. Immunodeficiency due to an early component of complement deficiency. | ||||||||||||
| PharmGKB | PA25903. | ||||||||||||
| GenAtlas | Search... | ||||||||||||
Phylogenomic databases | |||||||||||||
| HOVERGEN | HBG107123. | ||||||||||||
| InParanoid | P0C0L4. | ||||||||||||
| OrthoDB | EOG4JM7NW. | ||||||||||||
Enzyme and pathway databases | |||||||||||||
| Reactome | REACT_6900. Immune System. | ||||||||||||
Gene expression databases | |||||||||||||
| Genevestigator | P0C0L4. | ||||||||||||
| GermOnline | ENSG00000204319. Homo sapiens. | ||||||||||||
Family and domain databases | |||||||||||||
| InterPro | IPR009048. A-macroglobulin_rcpt-bd. IPR011626. A2M_comp. IPR002890. A2M_N. IPR011625. A2M_N_2. IPR000020. Anaphylatoxin/fibulin. IPR018081. Anaphylatoxin_. IPR001840. Anaphylatoxn. 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] | ||||||||||||
| Gene3D | G3DSA:2.60.40.690. A-macroglobulin_rcpt-bd. 1 hit. G3DSA:1.20.91.20. Anaphylatoxin. 1 hit. | ||||||||||||
| KO | K03989. | ||||||||||||
| Pfam | 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] | ||||||||||||
| PRINTS | PR00004. ANAPHYLATOXN. | ||||||||||||
| SMART | SM00104. ANATO. 1 hit. SM00643. C345C. 1 hit. [Graphical view] | ||||||||||||
| SUPFAM | SSF49410. AM_receptor_bind. 1 hit. SSF47686. Anaphylatoxin. 1 hit. SSF48239. Terp_cyc_toroid. 1 hit. SSF50242. TIMP_like. 1 hit. | ||||||||||||
| PROSITE | PS00477. ALPHA_2_MACROGLOBULIN. 1 hit. PS01177. ANAPHYLATOXIN_1. 1 hit. PS01178. ANAPHYLATOXIN_2. 1 hit. PS50189. NTR. 1 hit. [Graphical view] | ||||||||||||
| ProtoNet | Search... | ||||||||||||
Other | |||||||||||||
| NextBio | 2930. | ||||||||||||
| PMAP-CutDB | P0C0L4. | ||||||||||||
| SOURCE | Search... | ||||||||||||
Entry information
| Entry name | CO4A_HUMAN | ||||||||
| Accession | Primary (citable) accession number: P0C0L4 Secondary accession number(s): B0QZR6 Q9UIP5 | ||||||||
| Entry history |
| ||||||||
| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation program | Chordata Protein Annotation Program | ||||||||
| Disclaimer | Any 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. | ||||||||
Relevant documents
| Blood group antigen proteins Nomenclature of blood group antigens and list of entries |
| Human chromosome 6 Human chromosome 6: entries, gene names and cross-references to MIM |
| Human entries with polymorphisms or disease mutations List of human entries with polymorphisms or disease mutations |
| Human polymorphisms and disease mutations Index of human polymorphisms and disease mutations |
| MIM cross-references Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot |
| PDB cross-references Index of Protein Data Bank (PDB) cross-references |
| SIMILARITY comments Index of protein domains and families |

Clusters with