View source for Complement component 1r

{{Short description|Protein-coding gene in humans}}
{{cs1 config|name-list-style=vanc}}{{Infobox_gene}}
'''Complement C1r subcomponent''' ({{EC number|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]].<ref>{{cite book | vauthors = Sim RB | chapter = The human complement system serine proteases and their proenzymes | title = Proteolytic Enzymes, Part C | series = Methods in Enzymology | volume = 80 Pt C | pages = 26–42 | year = 1981 | pmid = 6281620 | doi = 10.1016/s0076-6879(81)80006-7 | isbn = 9780121819804 }}</ref><ref>{{cite journal | vauthors = Leytus SP, Kurachi K, Sakariassen KS, Davie EW | title = Nucleotide sequence of the cDNA coding for human complement C1r | journal = Biochemistry | volume = 25 | issue = 17 | pages = 4855–63 | date = August 1986 | pmid = 3021205 | doi = 10.1021/bi00365a020 }}</ref><ref>{{cite journal | vauthors = Müller-Eberhard HJ | title = Molecular organization and function of the complement system | journal = Annual Review of Biochemistry | volume = 57 | pages = 321–47 | year = 1988 | pmid = 3052276 | doi = 10.1146/annurev.bi.57.070188.001541 }}</ref> In humans, C1r is encoded by the ''C1R'' [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: C1R complement component 1, r subcomponent| url = https://www.ncbi.nlm.nih.gov/gene?Db=gene&Cmd=ShowDetailView&TermToSearch=715}}</ref>

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 [[proteolysis|proteolytic]] cleavage.

== Clinical significance ==
*[[Ehlers–Danlos syndromes|Ehlers–Danlos syndrome]] Periodontal type is associated with mutations in the ''CR1'' [[gene]]

== Function ==

C1r has been shown to [[Protein-protein interaction|interact]] with [[C1s]]. C1r [[proteolysis|cleaves]] C1s to form the active form of C1s.<ref name=pmid10092586>{{cite journal | vauthors = Thielens NM, Enrie K, Lacroix M, Jaquinod M, Hernandez JF, Esser AF, Arlaud GJ | title = 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 | journal = The Journal of Biological Chemistry | volume = 274 | issue = 14 | pages = 9149–59 | date = April 1999 | pmid = 10092586 | doi = 10.1074/jbc.274.14.9149 | doi-access = free }}</ref><ref name=pmid10878362>{{cite journal | vauthors = Thiel S, Petersen SV, Vorup-Jensen T, Matsushita M, Fujita T, Stover CM, Schwaeble WJ, Jensenius JC | title = 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 = Journal of Immunology | volume = 165 | issue = 2 | pages = 878–87 | date = July 2000 | pmid = 10878362 | doi = 10.4049/jimmunol.165.2.878 | doi-access = free }}</ref>

== References ==
{{reflist}}

== Further reading ==
{{refbegin | 2}}
* {{cite journal | vauthors = Lee SL, Wallace SL, Barone R, Blum L, Chase PH | title = Familial deficiency of two subunits of the first component of complement. C1r and C1s associated with a lupus erythematosus-like disease | journal = Arthritis and Rheumatism | volume = 21 | issue = 8 | pages = 958–67 | year = 1979 | pmid = 737019 | doi = 10.1002/art.1780210813 | doi-access =  }}
* {{cite journal | vauthors = Ward SL, Ingham KC | title = A calcium-binding monoclonal antibody that recognizes a non-calcium-binding epitope in the short consensus repeat units (SCRs) of complement C1r | journal = Molecular Immunology | volume = 29 | issue = 1 | pages = 83–93 | date = January 1992 | pmid = 1370572 | doi = 10.1016/0161-5890(92)90160-Y }}
* {{cite journal | vauthors = Luo C, Thielens NM, Gagnon J, Gal P, Sarvari M, Tseng Y, Tosi M, Zavodszky P, Arlaud GJ, Schumaker VN | title = 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 | journal = Biochemistry | volume = 31 | issue = 17 | pages = 4254–62 | date = May 1992 | pmid = 1533159 | doi = 10.1021/bi00132a015 }}
* {{cite journal | vauthors = Busby TF, Ingham KC | title = NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with C1q | journal = Biochemistry | volume = 29 | issue = 19 | pages = 4613–8 | date = May 1990 | pmid = 2372546 | doi = 10.1021/bi00471a016 }}
* {{cite journal | vauthors = Lyons LA, Kamboh MI, Ferrell RE | title = Genetic studies of low-abundance human plasma proteins. XI. Linkage analysis and population genetics of the C1S subcomponent of the first complement component | journal = Complement and Inflammation | volume = 6 | issue = 2 | pages = 81–7 | year = 1989 | pmid = 2541966 | doi =  10.1159/000463078}}
* {{cite journal | vauthors = Arlaud GJ, Van Dorsselaer A, Bell A, Mancini M, Aude C, Gagnon J | title = Identification of erythro-beta-hydroxyasparagine in the EGF-like domain of human C1r | journal = FEBS Letters | volume = 222 | issue = 1 | pages = 129–34 | date = September 1987 | pmid = 2820791 | doi = 10.1016/0014-5793(87)80205-3 | s2cid = 36194093 | doi-access = free | bibcode = 1987FEBSL.222..129A }}
* {{cite journal | vauthors = Nguyen VC, Tosi M, Gross MS, Cohen-Haguenauer O, Jegou-Foubert C, de Tand MF, Meo T, Frézal J | title = Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13 | journal = Human Genetics | volume = 78 | issue = 4 | pages = 363–8 | date = April 1988 | pmid = 2834284 | doi = 10.1007/BF00291737 | s2cid = 30582112 }}
* {{cite journal | vauthors = Leytus SP, Kurachi K, Sakariassen KS, Davie EW | title = Nucleotide sequence of the cDNA coding for human complement C1r | journal = Biochemistry | volume = 25 | issue = 17 | pages = 4855–63 | date = August 1986 | pmid = 3021205 | doi = 10.1021/bi00365a020 }}
* {{cite journal | vauthors = Journet A, Tosi M | title = Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r | journal = The Biochemical Journal | volume = 240 | issue = 3 | pages = 783–7 | date = December 1986 | pmid = 3030286 | pmc = 1147487 | doi =  10.1042/bj2400783}}
* {{cite journal | vauthors = Arlaud GJ, Willis AC, Gagnon J | title = Complete amino acid sequence of the A chain of human complement-classical-pathway enzyme C1r | journal = The Biochemical Journal | volume = 241 | issue = 3 | pages = 711–20 | date = February 1987 | pmid = 3036070 | pmc = 1147622 | doi =  10.1042/bj2410711}}
* {{cite journal | vauthors = Chesne S, Villiers CL, Arlaud GJ, Lacroix MB, Colomb MG | title = Fluid-phase interaction of C1 inhibitor (C1 Inh) and the subcomponents C1r and C1s of the first component of complement, C1 | journal = The Biochemical Journal | volume = 201 | issue = 1 | pages = 61–70 | date = January 1982 | pmid = 6282262 | pmc = 1163609 | doi =  10.1042/bj2010061}}
* {{cite journal | vauthors = Arlaud GJ, Gagnon J | title = Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r | journal = Biochemistry | volume = 22 | issue = 8 | pages = 1758–64 | date = April 1983 | pmid = 6303394 | doi = 10.1021/bi00277a003 }}
* {{cite journal | vauthors = Maruyama K, Sugano S | title = Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides | journal = Gene | volume = 138 | issue = 1–2 | pages = 171–4 | date = January 1994 | pmid = 8125298 | doi = 10.1016/0378-1119(94)90802-8 }}
* {{cite journal | vauthors = Nöthen MM, Dewald G | title = A common amino acid polymorphism in complement component C1R | journal = Human Molecular Genetics | volume = 3 | issue = 1 | pages = 217 | date = January 1994 | pmid = 8162045 | doi = 10.1093/hmg/3.1.217-a }}
* {{cite journal | vauthors = Gasque P, Ischenko A, Legoedec J, Mauger C, Schouft MT, Fontaine M | title = Expression of the complement classical pathway by human glioma in culture. A model for complement expression by nerve cells | journal = The Journal of Biological Chemistry | volume = 268 | issue = 33 | pages = 25068–74 | date = November 1993 | doi = 10.1016/S0021-9258(19)74572-4 | pmid = 8227070 | doi-access = free }}
* {{cite journal | vauthors = Pelloux S, Thielens NM, Hudry-Clergeon G, Pétillot Y, Filhol O, Arlaud GJ | title = Identification of a cryptic protein kinase CK2 phosphorylation site in human complement protease Clr, and its use to probe intramolecular interaction | journal = FEBS Letters | volume = 386 | issue = 1 | pages = 15–20 | date = May 1996 | pmid = 8635594 | doi = 10.1016/0014-5793(96)00403-6 | s2cid = 28372780 | doi-access = free | bibcode = 1996FEBSL.386...15P }}
* {{cite journal | vauthors = Bradley K, North J, Saunders D, Schwaeble W, Jeziorska M, Woolley DE, Whaley K | title = Synthesis of classical pathway complement components by chondrocytes | journal = Immunology | volume = 88 | issue = 4 | pages = 648–56 | date = August 1996 | pmid = 8881771 | pmc = 1456645 }}
* {{cite journal | vauthors = Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens NM, Gagnon J, Arlaud GJ | title = Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling | journal = Biochemistry | volume = 36 | issue = 21 | pages = 6270–82 | date = May 1997 | pmid = 9174342 | doi = 10.1021/bi962719i }}
* {{cite journal | vauthors = Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S | title = Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library | journal = Gene | volume = 200 | issue = 1–2 | pages = 149–56 | date = October 1997 | pmid = 9373149 | doi = 10.1016/S0378-1119(97)00411-3 }}
* {{cite journal | vauthors = Bersch B, Hernandez JF, Marion D, Arlaud GJ | title = Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, an atypical member of the EGF family | journal = Biochemistry | volume = 37 | issue = 5 | pages = 1204–14 | date = February 1998 | pmid = 9477945 | doi = 10.1021/bi971851v }}
{{refend}}

== External links ==
* {{UCSC gene info|C1R}}
* {{MeshName|Complement+C1r}}

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{{Complement system}}
{{Serine endopeptidases}}
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[[Category:Complement system]]
[[Category:EC 3.4.21]]


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