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.
^ abcGRCm38: Ensembl release 89: ENSMUSG00000098470 - Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^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. ISBN9780121819804. PMID 6281620.
^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.
^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.
^"Entrez Gene: C1R complement component 1, r subcomponent".
^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.
^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 readingedit
Lee SL, Wallace SL, Barone R, Blum L, Chase PH (1979). "Familial deficiency of two subunits of the first component of complement. C1r and C1s associated with a lupus erythematosus-like disease". Arthritis and Rheumatism. 21 (8): 958–67. doi:10.1002/art.1780210813. PMID 737019.
Ward SL, Ingham KC (January 1992). "A calcium-binding monoclonal antibody that recognizes a non-calcium-binding epitope in the short consensus repeat units (SCRs) of complement C1r". Molecular Immunology. 29 (1): 83–93. doi:10.1016/0161-5890(92)90160-Y. PMID 1370572.
Luo C, Thielens NM, Gagnon J, Gal P, Sarvari M, Tseng Y, Tosi M, Zavodszky P, Arlaud GJ, Schumaker VN (May 1992). "Recombinant human complement subcomponent C1s lacking beta-hydroxyasparagine, sialic acid, and one of its two carbohydrate chains still reassembles with C1q and C1r to form a functional C1 complex". Biochemistry. 31 (17): 4254–62. doi:10.1021/bi00132a015. PMID 1533159.
Busby TF, Ingham KC (May 1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with C1q". Biochemistry. 29 (19): 4613–8. doi:10.1021/bi00471a016. PMID 2372546.
Lyons LA, Kamboh MI, Ferrell RE (1989). "Genetic studies of low-abundance human plasma proteins. XI. Linkage analysis and population genetics of the C1S subcomponent of the first complement component". Complement and Inflammation. 6 (2): 81–7. doi:10.1159/000463078. PMID 2541966.
Arlaud GJ, Van Dorsselaer A, Bell A, Mancini M, Aude C, Gagnon J (September 1987). "Identification of erythro-beta-hydroxyasparagine in the EGF-like domain of human C1r". FEBS Letters. 222 (1): 129–34. doi:10.1016/0014-5793(87)80205-3. PMID 2820791. S2CID 36194093.
Nguyen VC, Tosi M, Gross MS, Cohen-Haguenauer O, Jegou-Foubert C, de Tand MF, Meo T, Frézal J (April 1988). "Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13". Human Genetics. 78 (4): 363–8. doi:10.1007/BF00291737. PMID 2834284. S2CID 30582112.
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.
Journet A, Tosi M (December 1986). "Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r". The Biochemical Journal. 240 (3): 783–7. doi:10.1042/bj2400783. PMC1147487. PMID 3030286.
Arlaud GJ, Willis AC, Gagnon J (February 1987). "Complete amino acid sequence of the A chain of human complement-classical-pathway enzyme C1r". The Biochemical Journal. 241 (3): 711–20. doi:10.1042/bj2410711. PMC1147622. PMID 3036070.
Chesne S, Villiers CL, Arlaud GJ, Lacroix MB, Colomb MG (January 1982). "Fluid-phase interaction of C1 inhibitor (C1 Inh) and the subcomponents C1r and C1s of the first component of complement, C1". The Biochemical Journal. 201 (1): 61–70. doi:10.1042/bj2010061. PMC1163609. PMID 6282262.
Arlaud GJ, Gagnon J (April 1983). "Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r". Biochemistry. 22 (8): 1758–64. doi:10.1021/bi00277a003. PMID 6303394.
Maruyama K, Sugano S (January 1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
Nöthen MM, Dewald G (January 1994). "A common amino acid polymorphism in complement component C1R". Human Molecular Genetics. 3 (1): 217. doi:10.1093/hmg/3.1.217-a. PMID 8162045.
Gasque P, Ischenko A, Legoedec J, Mauger C, Schouft MT, Fontaine M (November 1993). "Expression of the complement classical pathway by human glioma in culture. A model for complement expression by nerve cells". The Journal of Biological Chemistry. 268 (33): 25068–74. doi:10.1016/S0021-9258(19)74572-4. PMID 8227070.
Pelloux S, Thielens NM, Hudry-Clergeon G, Pétillot Y, Filhol O, Arlaud GJ (May 1996). "Identification of a cryptic protein kinase CK2 phosphorylation site in human complement protease Clr, and its use to probe intramolecular interaction". FEBS Letters. 386 (1): 15–20. doi:10.1016/0014-5793(96)00403-6. PMID 8635594. S2CID 28372780.
Bradley K, North J, Saunders D, Schwaeble W, Jeziorska M, Woolley DE, Whaley K (August 1996). "Synthesis of classical pathway complement components by chondrocytes". Immunology. 88 (4): 648–56. PMC1456645. PMID 8881771.
Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens NM, Gagnon J, Arlaud GJ (May 1997). "Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling". Biochemistry. 36 (21): 6270–82. doi:10.1021/bi962719i. PMID 9174342.
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (October 1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
Bersch B, Hernandez JF, Marion D, Arlaud GJ (February 1998). "Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, an atypical member of the EGF family". Biochemistry. 37 (5): 1204–14. doi:10.1021/bi971851v. PMID 9477945.
External linksedit
Human C1R genome location and C1R gene details page in the UCSC Genome Browser.
complement, component, complement, subcomponent, activated, complement, overbar, esterase, protein, involved, complement, system, innate, immune, system, humans, encoded, gene, c1ravailable, structurespdbortholog, search, pdbe, rcsblist, codes1apq, 1gpz, 1md7,. 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 C1RAvailable structuresPDBOrtholog search PDBe RCSBList of PDB id codes1APQ 1GPZ 1MD7 1MD8 2QY0IdentifiersAliasesC1R complement C1r EDSPD1External IDsOMIM 613785 MGI 3779804 HomoloGene 1313 GeneCards C1RGene location Human Chr Chromosome 12 human 1 Band12p13 31Start7 080 214 bp 1 End7 092 540 bp 1 Gene location Mouse Chr Chromosome 6 mouse 2 Band6 F2 6Start124 547 253 bp 2 End124 558 130 bp 2 RNA expression patternBgeeHumanMouse ortholog Top expressed inright lobe of livergallbladderright coronary arterygastric mucosaleft uterine tubeleft coronary arteryurinary bladdercanal of the cervixtibial nervemyometriumTop expressed inwhite adipose tissueadrenal glandurinary bladderliverovaryskeletal muscle tissuespleenislet of LangerhanslungtesticleMore reference expression dataBioGPSn aGene ontologyMolecular functionpeptidase activity serine type peptidase activity serine type endopeptidase activity protein binding hydrolase activity calcium ion bindingCellular componentextracellular region blood microparticle extracellular exosome extracellular spaceBiological processimmune response proteolysis complement activation classical pathway immune system process innate immune response complement activation regulation of complement activation zymogen activationSources Amigo QuickGOOrthologsSpeciesHumanMouseEntrez715667277EnsemblENSG00000159403ENSG00000288512ENSMUSG00000098470UniProtP00736Q8CFG9RefSeq mRNA NM 001733NM 001354346NM 001113356RefSeq protein NP 001724NP 001341275NP 001106827Location UCSC Chr 12 7 08 7 09 MbChr 6 124 55 124 56 MbPubMed search 3 4 WikidataView Edit HumanView Edit MouseC1r 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 Contents 1 Clinical significance 2 Function 3 References 4 Further reading 5 External linksClinical significance editEhlers Danlos syndrome Periodontal type is associated with mutations in the CR1 geneFunction editC1r has been shown to interact with C1s C1r cleaves C1s to form the active form of C1s 9 10 References edit a b c ENSG00000288512 GRCh38 Ensembl release 89 ENSG00000159403 ENSG00000288512 Ensembl May 2017 a b c GRCm38 Ensembl release 89 ENSMUSG00000098470 Ensembl May 2017 Human PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Mouse PubMed Reference National Center for Biotechnology Information U S National Library of Medicine 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 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 Muller 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 Entrez Gene C1R complement component 1 r subcomponent 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 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 editLee SL Wallace SL Barone R Blum L Chase PH 1979 Familial deficiency of two subunits of the first component of complement C1r and C1s associated with a lupus erythematosus like disease Arthritis and Rheumatism 21 8 958 67 doi 10 1002 art 1780210813 PMID 737019 Ward SL Ingham KC January 1992 A calcium binding monoclonal antibody that recognizes a non calcium binding epitope in the short consensus repeat units SCRs of complement C1r Molecular Immunology 29 1 83 93 doi 10 1016 0161 5890 92 90160 Y PMID 1370572 Luo C Thielens NM Gagnon J Gal P Sarvari M Tseng Y Tosi M Zavodszky P Arlaud GJ Schumaker VN May 1992 Recombinant human complement subcomponent C1s lacking beta hydroxyasparagine sialic acid and one of its two carbohydrate chains still reassembles with C1q and C1r to form a functional C1 complex Biochemistry 31 17 4254 62 doi 10 1021 bi00132a015 PMID 1533159 Busby TF Ingham KC May 1990 NH2 terminal calcium binding domain of human complement C1s mediates the interaction of C1r with C1q Biochemistry 29 19 4613 8 doi 10 1021 bi00471a016 PMID 2372546 Lyons LA Kamboh MI Ferrell RE 1989 Genetic studies of low abundance human plasma proteins XI Linkage analysis and population genetics of the C1S subcomponent of the first complement component Complement and Inflammation 6 2 81 7 doi 10 1159 000463078 PMID 2541966 Arlaud GJ Van Dorsselaer A Bell A Mancini M Aude C Gagnon J September 1987 Identification of erythro beta hydroxyasparagine in the EGF like domain of human C1r FEBS Letters 222 1 129 34 doi 10 1016 0014 5793 87 80205 3 PMID 2820791 S2CID 36194093 Nguyen VC Tosi M Gross MS Cohen Haguenauer O Jegou Foubert C de Tand MF Meo T Frezal J April 1988 Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13 Human Genetics 78 4 363 8 doi 10 1007 BF00291737 PMID 2834284 S2CID 30582112 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 Journet A Tosi M December 1986 Cloning and sequencing of full length cDNA encoding the precursor of human complement component C1r The Biochemical Journal 240 3 783 7 doi 10 1042 bj2400783 PMC 1147487 PMID 3030286 Arlaud GJ Willis AC Gagnon J February 1987 Complete amino acid sequence of the A chain of human complement classical pathway enzyme C1r The Biochemical Journal 241 3 711 20 doi 10 1042 bj2410711 PMC 1147622 PMID 3036070 Chesne S Villiers CL Arlaud GJ Lacroix MB Colomb MG January 1982 Fluid phase interaction of C1 inhibitor C1 Inh and the subcomponents C1r and C1s of the first component of complement C1 The Biochemical Journal 201 1 61 70 doi 10 1042 bj2010061 PMC 1163609 PMID 6282262 Arlaud GJ Gagnon J April 1983 Complete amino acid sequence of the catalytic chain of human complement subcomponent C1 r Biochemistry 22 8 1758 64 doi 10 1021 bi00277a003 PMID 6303394 Maruyama K Sugano S January 1994 Oligo capping a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides Gene 138 1 2 171 4 doi 10 1016 0378 1119 94 90802 8 PMID 8125298 Nothen MM Dewald G January 1994 A common amino acid polymorphism in complement component C1R Human Molecular Genetics 3 1 217 doi 10 1093 hmg 3 1 217 a PMID 8162045 Gasque P Ischenko A Legoedec J Mauger C Schouft MT Fontaine M November 1993 Expression of the complement classical pathway by human glioma in culture A model for complement expression by nerve cells The Journal of Biological Chemistry 268 33 25068 74 doi 10 1016 S0021 9258 19 74572 4 PMID 8227070 Pelloux S Thielens NM Hudry Clergeon G Petillot Y Filhol O Arlaud GJ May 1996 Identification of a cryptic protein kinase CK2 phosphorylation site in human complement protease Clr and its use to probe intramolecular interaction FEBS Letters 386 1 15 20 doi 10 1016 0014 5793 96 00403 6 PMID 8635594 S2CID 28372780 Bradley K North J Saunders D Schwaeble W Jeziorska M Woolley DE Whaley K August 1996 Synthesis of classical pathway complement components by chondrocytes Immunology 88 4 648 56 PMC 1456645 PMID 8881771 Lacroix M Rossi V Gaboriaud C Chevallier S Jaquinod M Thielens NM Gagnon J Arlaud GJ May 1997 Structure and assembly of the catalytic region of human complement protease C1r a three dimensional model based on chemical cross linking and homology modeling Biochemistry 36 21 6270 82 doi 10 1021 bi962719i PMID 9174342 Suzuki Y Yoshitomo Nakagawa K Maruyama K Suyama A Sugano S October 1997 Construction and characterization of a full length enriched and a 5 end enriched cDNA library Gene 200 1 2 149 56 doi 10 1016 S0378 1119 97 00411 3 PMID 9373149 Bersch B Hernandez JF Marion D Arlaud GJ February 1998 Solution structure of the epidermal growth factor EGF like module of human complement protease C1r an atypical member of the EGF family Biochemistry 37 5 1204 14 doi 10 1021 bi971851v PMID 9477945 External links editHuman C1R genome location and C1R gene details page in the UCSC Genome Browser Complement C1r at the U S National Library of Medicine Medical Subject Headings MeSH Portal nbsp Biology nbsp This article on a gene on human chromosome 12 is a stub You can help Wikipedia by expanding it vte nbsp This protein related article is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title Complement component 1r amp oldid 1193331780, wikipedia, wiki, book, books, library,
