C5
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesC5, C5D, C5a, C5b, CPAMD4, ECLZB, complement component 5, complement C5
External IDsOMIM: 120900 MGI: 96031 HomoloGene: 20412 GeneCards: C5
Orthologs
SpeciesHumanMouse
Entrez

727

15139

Ensembl

ENSG00000106804

ENSMUSG00000026874

UniProt

P01031

P06684

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 MbChr 2: 34.87 – 34.95 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/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.

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
  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.


This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.