Complement component 1r

Complement C1r subcomponent (EC 3.4.21.41, activated complement C1r, C overbar 1r esterase, C1r) is a protein involved in the complement system of the innate immune system.[5][6][7] In humans, C1r is encoded by the C1R gene.[8]

C1R
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesC1R, complement C1r, EDSPD1
External IDsOMIM: 613785 MGI: 3779804 HomoloGene: 1313 GeneCards: C1R
Orthologs
SpeciesHumanMouse
Entrez

715

667277

Ensembl

ENSG00000159403
ENSG00000288512

ENSMUSG00000098470

UniProt

P00736

Q8CFG9

RefSeq (mRNA)

NM_001733
NM_001354346

NM_001113356

RefSeq (protein)

NP_001724
NP_001341275

NP_001106827

Location (UCSC)Chr 12: 7.08 – 7.09 MbChr 6: 124.55 – 124.56 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme that activates C1s to its active form, by proteolytic cleavage.

Clinical significance

Function

C1r has been shown to interact with C1s. C1r cleaves C1s to form the active form of C1s.[9][10]

References

  1. ENSG00000288512 GRCh38: Ensembl release 89: ENSG00000159403, ENSG00000288512 - Ensembl, May 2017
  2. GRCm38: Ensembl release 89: ENSMUSG00000098470 - 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. Sim RB (1981). The human complement system serine proteases C1r and C1s and their proenzymes. Methods in Enzymology. Vol. 80 Pt C. pp. 26–42. doi:10.1016/s0076-6879(81)80006-7. PMID 6281620.
  6. Leytus SP, Kurachi K, Sakariassen KS, Davie EW (August 1986). "Nucleotide sequence of the cDNA coding for human complement C1r". Biochemistry. 25 (17): 4855–63. doi:10.1021/bi00365a020. PMID 3021205.
  7. Müller-Eberhard HJ (1988). "Molecular organization and function of the complement system". Annual Review of Biochemistry. 57: 321–47. doi:10.1146/annurev.bi.57.070188.001541. PMID 3052276.
  8. "Entrez Gene: C1R complement component 1, r subcomponent".
  9. Thielens NM, Enrie K, Lacroix M, Jaquinod M, Hernandez JF, Esser AF, Arlaud GJ (April 1999). "The N-terminal CUB-epidermal growth factor module pair of human complement protease C1r binds Ca2+ with high affinity and mediates Ca2+-dependent interaction with C1s". The Journal of Biological Chemistry. 274 (14): 9149–59. doi:10.1074/jbc.274.14.9149. PMID 10092586.
  10. Thiel S, Petersen SV, Vorup-Jensen T, Matsushita M, Fujita T, Stover CM, Schwaeble WJ, Jensenius JC (July 2000). "Interaction of C1q and mannan-binding lectin (MBL) with C1r, C1s, MBL-associated serine proteases 1 and 2, and the MBL-associated protein MAp19". Journal of Immunology. 165 (2): 878–87. doi:10.4049/jimmunol.165.2.878. PMID 10878362.

Further reading


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