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Complement component 1r

From Wikipedia, the free encyclopedia
Protein-coding gene in humans
C1R
Available structures
PDBOrtholog search:PDBeRCSB
List of PDB id codes

1APQ,1GPZ,1MD7,1MD8,2QY0

Identifiers
AliasesC1R, complement C1r, EDSPD1
External IDsOMIM:613785;MGI:3779804;HomoloGene:1313;GeneCards:C1R;OMA:C1R - orthologs
Gene location (Human)
Chromosome 12 (human)
Chr.Chromosome 12 (human)[1]
Chromosome 12 (human)
Genomic location for C1R
Genomic location for C1R
Band12p13.31Start7,080,214bp[1]
End7,092,540bp[1]
Gene location (Mouse)
Chromosome 6 (mouse)
Chr.Chromosome 6 (mouse)[2]
Chromosome 6 (mouse)
Genomic location for C1R
Genomic location for C1R
Band6 F2|6Start124,547,253bp[2]
End124,558,130bp[2]
RNA expression pattern
Bgee
HumanMouse (ortholog)
Top expressed in
  • right lobe of liver

  • right ovary

  • gallbladder

  • right coronary artery

  • gastric mucosa

  • left ovary

  • left uterine tube

  • left coronary artery

  • urinary bladder

  • canal of the cervix
Top expressed in
  • white adipose tissue

  • adrenal gland

  • muscle of thigh

  • urinary bladder

  • dentate gyrus of hippocampal formation granule cell

  • liver

  • ovary

  • skeletal muscle tissue

  • spleen

  • islet of Langerhans
More reference expression data
BioGPS
n/a
Gene ontology
Molecular function
Cellular component
Biological process
Sources:Amigo /QuickGO
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

Complement C1r subcomponent (EC3.4.21.41,activated complement C1r,C overbar 1r esterase,C1r) is aprotein involved in thecomplement system of theinnate immune system.[5][6][7] In humans, C1r is encoded by theC1Rgene.[8]

C1r along withC1q andC1s form theC1 complex, which is the first component of the serum complement system. C1r is anenzyme that activates C1s to its active form, byproteolytic cleavage.

Clinical significance

[edit]

Function

[edit]

C1r has been shown tointeract withC1s. C1rcleaves C1s to form the active form of C1s.[9][10]

References

[edit]
  1. ^abcENSG00000288512 GRCh38: Ensembl release 89: ENSG00000159403, ENSG00000288512Ensembl, May 2017
  2. ^abcGRCm38: Ensembl release 89: ENSMUSG00000098470Ensembl, 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 and their proenzymes".Proteolytic Enzymes, Part C. Methods in Enzymology. Vol. 80 Pt C. pp. 26–42.doi:10.1016/s0076-6879(81)80006-7.ISBN 9780121819804.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

[edit]

External links

[edit]
PDB gallery
  • 1apq: STRUCTURE OF THE EGF-LIKE MODULE OF HUMAN C1R, NMR, 19 STRUCTURES
    1apq: STRUCTURE OF THE EGF-LIKE MODULE OF HUMAN C1R, NMR, 19 STRUCTURES
  • 1gpz: THE CRYSTAL STRUCTURE OF THE ZYMOGEN CATALYTIC DOMAIN OF COMPLEMENT PROTEASE C1R
    1gpz: THE CRYSTAL STRUCTURE OF THE ZYMOGEN CATALYTIC DOMAIN OF COMPLEMENT PROTEASE C1R
  • 1md7: Monomeric structure of the zymogen of complement protease C1r
    1md7: Monomeric structure of the zymogen of complement protease C1r
  • 1md8: Monomeric structure of the active catalytic domain of complement protease C1r
    1md8: Monomeric structure of the active catalytic domain of complement protease C1r
Pathways
Activators/enzymes
Early
Middle
Late
Inhibitors
Complement receptors
Function
Digestive enzymes
Coagulation
Complement system
Otherimmune system
Venombin
Other
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