NP_000054.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
69,444 Da
NCBI Official Full Name
complement C2 isoform 1 preproprotein
NCBI Official Synonym Full Names
complement component 2
NCBI Official Synonym Symbols
NCBI Protein Information
complement C2
UniProt Protein Name
Complement C2
UniProt Synonym Protein Names
C3/C5 convertase
UniProt Entry Name
CO2_HUMAN
NCBI Summary for C2
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]
UniProt Comments for C2
C2: 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. Defects in C2 are the cause of complement component 2 deficiency (C2D). A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted; Secreted, signal peptide; Protease; EC 3.4.21.43
Chromosomal Location of Human Ortholog: 6p21.3
Cellular Component: extracellular space; extracellular region
Molecular Function: metal ion binding; serine-type endopeptidase activity
Biological Process: regulation of complement activation; innate immune response; proteolysis; complement activation, classical pathway; response to nutrient; complement activation
Disease: Macular Degeneration, Age-related, 14; Complement Component 2 Deficiency
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Products associated with C2 sirna
Pathways associated with C2 sirna
Diseases associated with C2 sirna
Organs/Tissues associated with C2 sirna
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