C2 (complement C2)

Gene

• Entrez ID: 717
• Gene name: Complement C2
• Gene symbol: C2
• Synonyms: ARMD14, CO2
• Chromosome: 6
• Chromosome location: 6p21.33
• Summary: Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. Activated C1 cleaves C2 into C2a and C2b. The serine proteinase C2a then combines with complement factor 4b to create the C3 or C5 convertase. Deficiency of C2 has been reported to associated with certain autoimmune diseases and SNPs in this gene have been associated with altered susceptibility to age-related macular degeneration. This gene localizes within the class III region of the MHC on the short arm of chromosome 6. Alternative splicing results in multiple transcript variants encoding distinct isoforms. Additional transcript variants have been described in publications but their full-length sequence has not been determined.[provided by RefSeq, Mar 2009]

miRNA

miRTarBase ID	miRNA	Experiments	Reference
MIRT017726	hsa-miR-335-5p	Microarray	18185580
MIRT021561	hsa-miR-142-3p	Microarray	17612493

Gene ontology (GO)

GO ID	Ontology	Definition	Evidence	Reference
GO:0004252	Function	Serine-type endopeptidase activity	IEA
GO:0005515	Function	Protein binding	IPI	25910212, 32296183
GO:0005576	Component	Extracellular region	TAS
GO:0005615	Component	Extracellular space	TAS	8621452
GO:0006508	Process	Proteolysis	IEA
GO:0006956	Process	Complement activation	IMP	19302245
GO:0006956	Process	Complement activation	TAS
GO:0006958	Process	Complement activation, classical pathway	IEA
GO:0007584	Process	Response to nutrient	IEA
GO:0030449	Process	Regulation of complement activation	TAS
GO:0045087	Process	Innate immune response	IEA
GO:0046872	Function	Metal ion binding	IEA
GO:0070062	Component	Extracellular exosome	HDA	23533145
GO:2000427	Process	Positive regulation of apoptotic cell clearance	IMP	19302245

Other IDs

• MIM: 613927
• HGNC: 1248
• e!Ensembl: ENSG00000166278

Protein

• UniProt ID: P06681
• Protein name: Complement C2 (EC 3.4.21.43) (C3/C5 convertase) [Cleaved into: Complement C2b fragment; Complement C2a fragment]
• Protein function: Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor C4b to generate the C3 or C5 convertase.
• PDB: 2I6Q, 2I6S, 2ODP, 2ODQ, 3ERB
• Family and domains:

  Pfam

  Accession	ID	Position in sequence	Description	Type
  PF00084	Sushi	89 → 144	Sushi repeat (SCR repeat)	Domain
  PF00084	Sushi	151 → 204	Sushi repeat (SCR repeat)	Domain
  PF00092	VWA	254 → 451	von Willebrand factor type A domain	Domain
  PF00089	Trypsin	466 → 711	Trypsin	Domain

• Sequence:

  MGPLMVLFCLLFLYPGLADSAPSCPQNVNISGGTFTLSHGWAPGSLLTYSCPQGLYPSPA
  SRLCKSSGQWQTPGATRSLSKAVCKPVRCPAPVSFENGIYTPRLGSYPVGGNVSFECEDG
  FILRGSPVRQCRPNGMWDGETAVCDNGAGHCPNPGISLGAVRTGFRFGHGDKVRYRCSSN
  LVLTGSSERECQGNGVWSGTEPICRQPYSYDFPEDVAPALGTSFSHMLGATNPTQKTKES
  LGRKIQIQRSGHLNLYLLLDCSQSVSENDFLIFKESASLMVDRIFSFEINVSVAIITFAS
  EPKVLMSVLNDNSRDMTEVISSLENANYKDHENGTGTNTYAALNSVYLMMNNQMRLLGME
  TMAWQEIRHAIILLTDGKSNMGGSPKTAVDHIREILNINQKRNDYLDIYAIGVGKLDVDW
  RELNELGSKKDGERHAFILQDTKALHQVFEHMLDVSKLTDTICGVGNMSANASDQERTPW
  HVTIKPKSQETCRGALISDQWVLTAAHCFRDGNDHSLWRVNVGDPKSQWGKEFLIEKAVI
  SPGFDVFAKKNQGILEFYGDDIALLKLAQKVKMSTHARPICLPCTMEANLALRRPQGSTC
  RDHENELLNKQSVPAHFVALNGSKLNINLKMGVEWTSCAEVVSQEKTMFPNLTDVREVVT
  DQFLCSGTQEDESPCKGESGGAVFLERRFRFFQVGLVSWGLYNPCLGSADKNSRKRAPRS
  KVPPPRDFHINLFRMQPWLRQHLGDVLNFLPL

• Sequence length: 752