C1r (complemento)

C1R
Estruturas dispoñibles
PDB	Buscar ortólogos: PDBe, RCSB Lista de códigos PDB 1APQ, 1GPZ, 1MD7, 1MD8, 2QY0 ;
Identificadores
Nomenclatura	Outros nomes C1R, complemento C1r, EDSPD1 ;
Símbolo	C1R (HGNC: 1246)
Identificadores; externos	OMIM: 613785 ; MGI: 3779804 ; HomoloGene: 1313 ; EBI: C1R; GeneCards: Xene C1R; UniProt: C1R;
Locus	Cr. 12 p13.31
Ontoloxía Xénica	Referencias: AmiGO / QuickGO
	Referencias: AmiGO / QuickGO
Ortólogos
Especies
Humano	Rato
Entrez
715	667277
Ensembl
Véxase HS	Véxase MM
UniProt
P00736	Q8CFG9
RefSeq; (ARNm)
NM_001733	NM_001113356
RefSeq; (proteína) NCBI
NP_001724	NP_001106827
Localización (UCSC)
Cr. 12:; 7.08 – 7.09 Mb	Cr. 6:; 124.55 – 124.56 Mb
PubMed (Busca)
715 ;	667277

O compoñente do complemento 1r (EC 3.4.21.41), complemento C1r ou C1r) é unha proteína que forma parte do sistema do complemento do sistema inmunitario innato.^[1]^[2]^[3] Nos humanos C1r está codificado no xene C1R do cromosoma 12.^[4]

C1r, xunto con C1q e C1s, forma o complexo C1, que é o primeiro compoñente do sistema do complemento do soro sanguíneo. C1r é un encima que transforma C1s na súa forma activa, por medio dun corte proteolítico.

Importancia clínica

A síndrome de Ehlers-Danlos de tipo periodontal está asociada con mutacións no xene CR1.

Función

O C1r presenta interaccións con C1s. C1r corta proteoliticamente C1s para orixinar a forma activa de C1s.^[5]^[6]

Notas

↑ Sim RB (1981). "The human complement system serine proteases and their proenzymes". Proteolytic Enzymes, Part C. Methods in Enzymology. 80 Pt C. pp. 26–42. ISBN 9780121819804. PMID 6281620. doi:10.1016/s0076-6879(81)80006-7.
↑ Leytus SP, Kurachi K, Sakariassen KS, Davie EW (agosto de 1986). "Nucleotide sequence of the cDNA coding for human complement C1r". Biochemistry 25 (17): 4855–63. PMID 3021205. doi:10.1021/bi00365a020.
↑ Müller-Eberhard HJ (1988). "Molecular organization and function of the complement system". Annual Review of Biochemistry 57: 321–47. PMID 3052276. doi:10.1146/annurev.bi.57.070188.001541.
↑ "Entrez Gene: C1R complement component 1, r subcomponent".
↑ Thielens NM, Enrie K, Lacroix M, Jaquinod M, Hernandez JF, Esser AF, Arlaud GJ (abril de 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. PMID 10092586. doi:10.1074/jbc.274.14.9149.
↑ Thiel S, Petersen SV, Vorup-Jensen T, Matsushita M, Fujita T, Stover CM, Schwaeble WJ, Jensenius JC (xullo de 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. PMID 10878362. doi:10.4049/jimmunol.165.2.878.

Véxase tamén

Outros artigos

C1q - outro membro do complexo C1
C1s - outro membro do complexo C1

Bibliografía

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. PMID 737019. doi:10.1002/art.1780210813.
Ward SL, Ingham KC (xaneiro de 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. PMID 1370572. doi:10.1016/0161-5890(92)90160-Y.
Luo C, Thielens NM, Gagnon J, Gal P, Sarvari M, Tseng Y, Tosi M, Zavodszky P, Arlaud GJ, Schumaker VN (maio de 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. PMID 1533159. doi:10.1021/bi00132a015.
Busby TF, Ingham KC (maio de 1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with C1q". Biochemistry 29 (19): 4613–8. PMID 2372546. doi:10.1021/bi00471a016.
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. PMID 2541966. doi:10.1159/000463078.
Arlaud GJ, Van Dorsselaer A, Bell A, Mancini M, Aude C, Gagnon J (setembro de 1987). "Identification of erythro-beta-hydroxyasparagine in the EGF-like domain of human C1r". FEBS Letters 222 (1): 129–34. Bibcode:1987FEBSL.222..129A. PMID 2820791. doi:10.1016/0014-5793(87)80205-3.
Nguyen VC, Tosi M, Gross MS, Cohen-Haguenauer O, Jegou-Foubert C, de Tand MF, Meo T, Frézal J (abril de 1988). "Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13". Human Genetics 78 (4): 363–8. PMID 2834284. doi:10.1007/BF00291737.
Leytus SP, Kurachi K, Sakariassen KS, Davie EW (agosto de 1986). "Nucleotide sequence of the cDNA coding for human complement C1r". Biochemistry 25 (17): 4855–63. PMID 3021205. doi:10.1021/bi00365a020.
Journet A, Tosi M (decembro de 1986). "Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r". The Biochemical Journal 240 (3): 783–7. PMC 1147487. PMID 3030286. doi:10.1042/bj2400783.
Arlaud GJ, Willis AC, Gagnon J (febreiro de 1987). "Complete amino acid sequence of the A chain of human complement-classical-pathway enzyme C1r". The Biochemical Journal 241 (3): 711–20. PMC 1147622. PMID 3036070. doi:10.1042/bj2410711.
Chesne S, Villiers CL, Arlaud GJ, Lacroix MB, Colomb MG (xaneiro de 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. PMC 1163609. PMID 6282262. doi:10.1042/bj2010061.
Arlaud GJ, Gagnon J (abril de 1983). "Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r". Biochemistry 22 (8): 1758–64. PMID 6303394. doi:10.1021/bi00277a003.
Maruyama K, Sugano S (xaneiro de 1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene 138 (1–2): 171–4. PMID 8125298. doi:10.1016/0378-1119(94)90802-8.
Nöthen MM, Dewald G (xaneiro de 1994). "A common amino acid polymorphism in complement component C1R". Human Molecular Genetics 3 (1): 217. PMID 8162045. doi:10.1093/hmg/3.1.217-a.
Gasque P, Ischenko A, Legoedec J, Mauger C, Schouft MT, Fontaine M (novembro de 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. PMID 8227070. doi:10.1016/S0021-9258(19)74572-4.
Pelloux S, Thielens NM, Hudry-Clergeon G, Pétillot Y, Filhol O, Arlaud GJ (maio de 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. Bibcode:1996FEBSL.386...15P. PMID 8635594. doi:10.1016/0014-5793(96)00403-6.
Bradley K, North J, Saunders D, Schwaeble W, Jeziorska M, Woolley DE, Whaley K (agosto de 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 (maio de 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. PMID 9174342. doi:10.1021/bi962719i.
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (outubro de 1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene 200 (1–2): 149–56. PMID 9373149. doi:10.1016/S0378-1119(97)00411-3.
Bersch B, Hernandez JF, Marion D, Arlaud GJ (febreiro de 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. PMID 9477945. doi:10.1021/bi971851v.

Ligazóns externas

Localización no xenoma humano de C1R e páxina con detalles sobre o xene C1R no UCSC Genome Browser.
Complement C1r Medical Subject Headings (MeSH) na Biblioteca Nacional de Medicina dos EUA.

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[1] Sim RB (1981). "The human complement system serine proteases and their proenzymes". Proteolytic Enzymes, Part C. Methods in Enzymology. 80 Pt C. pp. 26–42. ISBN 9780121819804. PMID 6281620. doi:10.1016/s0076-6879(81)80006-7.

[2] Leytus SP, Kurachi K, Sakariassen KS, Davie EW (agosto de 1986). "Nucleotide sequence of the cDNA coding for human complement C1r". Biochemistry 25 (17): 4855–63. PMID 3021205. doi:10.1021/bi00365a020.

[3] Müller-Eberhard HJ (1988). "Molecular organization and function of the complement system". Annual Review of Biochemistry 57: 321–47. PMID 3052276. doi:10.1146/annurev.bi.57.070188.001541.

[entrez-4] "Entrez Gene: C1R complement component 1, r subcomponent".

[pmid10092586-5] Thielens NM, Enrie K, Lacroix M, Jaquinod M, Hernandez JF, Esser AF, Arlaud GJ (abril de 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. PMID 10092586. doi:10.1074/jbc.274.14.9149.

[pmid10878362-6] Thiel S, Petersen SV, Vorup-Jensen T, Matsushita M, Fujita T, Stover CM, Schwaeble WJ, Jensenius JC (xullo de 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. PMID 10878362. doi:10.4049/jimmunol.165.2.878.

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