Complement component 5

Last updated

C5
C5.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases C5 , C5D, C5a, C5b, CPAMD4, ECLZB, complement component 5, complement C5
External IDs OMIM: 120900; MGI: 96031; HomoloGene: 20412; GeneCards: C5; OMA:C5 - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001735
NM_001317163
NM_001317164

NM_010406

RefSeq (protein)

NP_001304092
NP_001304093
NP_001726

NP_034536

Location (UCSC) Chr 9: 120.93 – 121.08 Mb Chr 2: 34.87 – 34.95 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Complement component 5 is a protein that in humans is encoded by the C5 gene. [5]

Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b:

Deficiency is thought to cause Leiner's disease.

Function

Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. This protein is composed of alpha and beta polypeptide chains that are linked by a disulfide bridge. An activation peptide, C5a, which is an anaphylatoxin that possesses potent spasmogenic and chemotactic activity, is derived from the alpha polypeptide via cleavage with a C5-convertase. The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components. [5]

Clinical significance

Mutations in this gene cause complement component 5 deficiency, a disease where patients show a propensity for severe recurrent infections. Defects in this gene have also been linked to a susceptibility to liver fibrosis and to rheumatoid arthritis. [5]

Therapeutic applications

The drug eculizumab (trade name Soliris) prevents cleavage of C5 into C5a and C5b. [7]

Complement system pathway

Membrane attack complex. Formowanie MAC-en.svg
Membrane attack complex.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000106804 Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000026874 Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. 1 2 3 "C5 complement C5 (Homo sapiens (human)) Gene ID: 727". www.ncbi.nlm.nih.gov. 29 November 2020. Retrieved 9 December 2020.
  6. Immunology at MCG 1/phagocyt [ dead link ]
  7. Dubois E, Cohen A (2009). "Eculizumab". Br J Clin Pharmacol. 68 (3): 318–319. doi:10.1111/j.1365-2125.2009.03491.x. PMC   2766470 . PMID   19740388.

Further reading

This article incorporates text from the United States National Library of Medicine, which is in the public domain.