Complement 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.

Complement C2 Related Areas

Enzyme>>Protease & Regulator>>Serine Protease & Regulator>>Complement System>>Complement C2

Immunology>>Innate Immunity>>Complement System>>Complement C2

Complement C2 Alternative Names

C2 , DADB-122G4.1, CO2, DKFZp779M0311 [Homo sapiens]

C2 , RP24-317H19.3 [Mus musculus]

Summaries for Complement C2

Entrez Gene summary for Complement 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

OMIM - description for Complement C2 :

A number sign (#) is used with this entry because complement component-2 deficiency is caused by homozygous or compound heterozygous mutation in the C2 gene

Wikipedia summary for Complement C2 :

Complement C2 is a protein that in humans is encoded by the C2 gene.[1] The protein encoded by this gene is part of the classical pathway of complement system. Activated C1 cleaves C2 into C2a and C2b. C2a leads to activation of C3. Deficiency of C2 has been reported to associated with certain autoimmune diseases

Human Complement C2 Protein General Information

 

• Protein names: Recommended name: Complement C2
• Sequence length: 752 AA.
• Domain: Repeat Signal Sushi
• Sequence similarities: Belongs to the peptidase S1 family. Contains 1 peptidase S1 domain. Contains 3 Sushi (CCP/SCR) domains. Contains 1 VWFA domain.
• Subunit structure: C2a interacts with Schistosoma haematobium TOR (via N-terminal extracellular domain). This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection.
• Subcellular location: Secreted.
• Involvement in disease: Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000]. 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.

General information above from UniProt

Function for Complement C2 Protein

UniProtKB:

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.

Genatlas:

• serine protease/trypsin family
• Complement C2 peptidase S1 family
• Complement C2 is the part of the classical pathway of the complement system cleaved by activated factor C1 into two fragments : C2B and C2A

Homology for human Complement C2

• homolog to murine C2

Phenotype Information for Complement C2

• Gene/Locus: C2
• Phenotype: C2 deficiency {Macular degeneration, age-related, reduced risk of}

Phenotype Information for Complement C2 from OMIM (Online Mendelian Inheritance in Man)