Complement Component 7

Complement component 7 is a component of the complement system. It belongs to the complement C6/C7/C8/C9 family. It contains 1 EGF-like domain, 1 LDL-receptor class A domain, 1 MACPF domain, 2 Sushi (CCP/SCR) domains and 2 TSP type-1 domains. Complement component 7 serves as a membrane anchor. It participates in the formation of Membrane Attack Complex (MAC). People with C7 deficiency are prone to bacterial infection. It is a constituent of MAC that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. Defects in C7 are a cause of complement component 7 deficiency (C7D). A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.

Complement Component 7 Related Areas

Immunology>>Innate Immunity>>Complement System>> complement component 7

Complement Component 7 Alternative Names

complement component C7 [Homo sapiens]

A530026G17, Gm1238, complement component C7 [Mus musculus]

Summaries for Complement Component 7

Entrez Gene summary for C7:

Complement Component 7 is a component of the complement system. Complement Component 7 participates in the formation of Membrane Attack Complex (MAC). People with Complement Component 7 deficiency are prone to bacterial infection. [provided by RefSeq, Jul 2008]

OMIM - description for Complement Component 7:

Complement Component 7 is a single-chain plasma glycoprotein involved in the cytolytic phase of complement activation (Hobart et al., 1995). Mutations in the C7 gene cause C7 deficiency (610102), a defect associated with increased susceptibility to neisserial recurrent infections (Barroso et al., 2004).

Wikipedia summary for Complement Component 7:

Complement component 7 is a protein involved in the complement system.

Human Complement Component 7 Protein General Information

 

• Protein names: Complement component C7
• Sequence length: 843 AA.
• Sequence similarities: Complement Component 7 elongs to the complement C6/C7/C8/C9 family. Complement Component 7 contains 1 EGF-like domain. Complement Component 7 contains 1 LDL-receptor class A domain. C7 contains 1 MACPF domain. C7 contains 2 Sushi (CCP/SCR) domains. C7 contains 2 TSP type-1 domains.
• Post-translational modification: C7 has 28 disulfide bridges. O- and C-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans.
• Subunit structure: Monomer or dimer; as a C5b-7 complex it can also form multimeric rosettes. MAC assembly is initiated by protelytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and multiple copies of the pore-forming subunit C9.
• Subcellular location: Secreted.
• Involvement in disease: Defects in Complement Component 7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.

General information above from UniProt

Function for Complement Component 7 Protein

UniProtKB:

Complement Component 7 is constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C7 serves as a membrane anchor.

Genatlas:

• stable insertion of the C5b, C6, C7 complex into the cell membrane

Homology for human Complement Component 7

• ortholog to murine C7

Phenotype Information for Complement Component 7

• Gene/Locus: C7
• Phenotype: C7 deficiency

Phenotype Information for Complement Component 7 from OMIM (Online Mendelian Inheritance in Man)