C1R

Summary

Gene Symbol: C1R
Description: complement C1r
Alias: EDSPD1, complement C1r subcomponent, complement component 1, r subcomponent
Species: human
Products:     C1R

Top Publications

  1. Uekita T, Fujii S, Miyazawa Y, Hashiguchi A, Abe H, Sakamoto M, et al. Suppression of autophagy by CUB domain-containing protein 1 signaling is essential for anchorage-independent survival of lung cancer cells. Cancer Sci. 2013;104:865-70 pubmed publisher
    CUB (C1r/C1s, urchin embryonic growth factor, BMP1) domain-containing protein 1 (CDCP1) has been implicated in promoting metastasis of cancer cells through several mechanisms, including the inhibition of anoikis, which is cell death ..
  2. Inouye K, Tsuzuki S, Yasumoto M, Kojima K, Mochida S, Fushiki T. Identification of the matriptase second CUB domain as the secondary site for interaction with hepatocyte growth factor activator inhibitor type-1. J Biol Chem. 2010;285:33394-403 pubmed publisher
    ..region of matriptase comprises a noncatalytic stem domain (containing two tandem repeats of complement proteases C1r/C1s-urchin embryonic growth factor-bone morphogenetic protein (CUB) domain) and a catalytic serine protease domain...
  3. Li J, Zhang X, Xu J, Pei X, Wu Z, Wang T, et al. iTRAQ analysis of liver immune-related proteins from darkbarbel catfish (Pelteobagrus vachelli) infected with Edwardsiella ictaluri. Fish Shellfish Immunol. 2019;87:695-704 pubmed publisher
    ..The 10 immune-related proteins included complement component C1r, C3, C5, C7, and C9 and plasma protease C1 inhibitor (C1-INH), signal recognition particle 54 kDa protein (SRP54),..
  4. Chang C, Tung Y, Lin Y, Liao C, Chiu C, Tung T, et al. Effects of Caloric Restriction with Protein Supplementation on Plasma Protein Profiles in Middle-Aged Women with Metabolic Syndrome-A Preliminary Open Study. J Clin Med. 2019;8: pubmed publisher
    ..the relative plasma levels of alpha-2-macroglobulin (A2M), C4b-binding protein alpha chain (C4BPA), complement C1r subcomponent-like protein (C1RL), complement component C6 (C6), complement component C8 gamma chain (C8G), and ..
  5. Gorbushin A. Derivatives of the lectin complement pathway in Lophotrochozoa. Dev Comp Immunol. 2019;94:35-58 pubmed publisher
    ..the common ancestor of TEP-associated proteases (C2/Bf/Сf/Lf), the common ancestor of MASP-like proteases (MASP/C1r/C1s, MReM1/MReM2) and multimeric recognition proteins (C1q-, MBL- and FCN-homologs)...
  6. Kapferer Seebacher I, Waisfisz Q, Boesch S, Bronk M, van Tintelen P, Gizewski E, et al. Periodontal Ehlers-Danlos syndrome is associated with leukoencephalopathy. Neurogenetics. 2019;20:1-8 pubmed publisher
    ..Whole exome sequencing revealed heterozygous C1R mutations c.926G>T (p.Cys309Phe, Family A) and c.149_150TC>AT (p.Val50Asp, Family B)...
  7. Bourhis J, Vadon Le Goff S, Afrache H, Mariano N, Kronenberg D, Thielens N, et al. Procollagen C-proteinase enhancer grasps the stalk of the C-propeptide trimer to boost collagen precursor maturation. Proc Natl Acad Sci U S A. 2013;110:6394-9 pubmed publisher
    ..the trimer that are essential for binding, thus revealing structural parallels with the interactions of Complement C1r/C1s, Uegf, BMP-1 (CUB) domain-containing proteins in diverse biological systems such as complement activation, ..
  8. Loffredo S, Staiano R, Granata F, Costantino V, Borriello F, Frattini A, et al. Simplexide induces CD1d-dependent cytokine and chemokine production from human monocytes. PLoS ONE. 2014;9:e111326 pubmed publisher
    ..activation by simplexide and iii) simplexide induces cytokine production from CD1d-transfected but not parental C1R cell line. Finally, we have shown that simplexide also induces iNKT cell expansion in vitro...
  9. Föcking M, Sabherwal S, Cates H, Scaife C, Dicker P, Hryniewiecka M, et al. Complement pathway changes at age 12 are associated with psychotic experiences at age 18 in a longitudinal population-based study: evidence for a role of stress. Mol Psychiatry. 2019;: pubmed publisher
    ..of 11 complement proteins, including three that were altered in the same direction in individuals with PE (C1R↑, CFH↑, and C5↑)...

More Information

Publications115 found, 100 shown here

  1. Xiong F, Ge W, Ma C. Quantitative proteomics reveals distinct composition of amyloid plaques in Alzheimer's disease. Alzheimers Dement. 2019;15:429-440 pubmed publisher
    ..Intriguingly, proteins including synaptic structural proteins and complement C1r, C5, and C9 were found to be upregulated in AD APs but not non-AD APs...
  2. Keizer M, Pouw R, Kamp A, Patiwael S, Marsman G, Hart M, et al. TFPI inhibits lectin pathway of complement activation by direct interaction with MASP-2. Eur J Immunol. 2015;45:544-50 pubmed publisher
    ..TFPI as a novel selective inhibitor of MASP-2, without affecting MASP-1 or the classical pathway proteases C1s and C1r. Kunitz-2 domain of TFPI is required for the inhibition of MASP-2...
  3. Pompili E, Fabrizi C, Somma F, Correani V, Maras B, Schininà M, et al. PAR1 activation affects the neurotrophic properties of Schwann cells. Mol Cell Neurosci. 2017;79:23-33 pubmed publisher
    ..them (such as macrophage migration inhibitory factor, matrix metalloproteinase-2, decorin, syndecan 4, complement C1r subcomponent, angiogenic factor with G patch and FHA domains 1) appear to be transcriptionally regulated after PAR1 ..
  4. Bisschops L, Van Der Hoeven J, Mollnes T, Hoedemaekers C. Seventy-two hours of mild hypothermia after cardiac arrest is associated with a lowered inflammatory response during rewarming in a prospective observational study. Crit Care. 2014;18:546 pubmed publisher
    ..IL-8 and monocyte chemotactic protein 1), intercellular adhesion molecule 1 and complement activation products (C1r-C1s-C1inhibitor, C4bc, C3bPBb, C3bc and terminal complement complex) were measured...
  5. Malfait F. Vascular aspects of the Ehlers-Danlos Syndromes. Matrix Biol. 2018;71-72:380-395 pubmed publisher
    ..sulfate epimerase (DSE)), (putative) transcription factors (ZNF469, PRDM5), components of the complement pathway (C1r, C1s) and an intracellular Zinc transporter (ZIP13)...
  6. Kitamura Y, Kojima M, Kurosawa T, Sasaki R, Ichihara S, Hiraku Y, et al. Proteomic Profiling of Exosomal Proteins for Blood-based Biomarkers in Parkinson's Disease. Neuroscience. 2018;392:121-128 pubmed publisher
    ..The expression levels of three proteins (clusterin, complement C1r subcomponent, and apolipoprotein A1) in PD patients at HY stages II and III were significantly decreased compared to ..
  7. Wallis R, Mitchell D, Schmid R, Schwaeble W, Keeble A. Paths reunited: Initiation of the classical and lectin pathways of complement activation. Immunobiology. 2010;215:1-11 pubmed publisher
    ..knowledge is still incomplete, especially with regard to the interactions between its subcomponents C1q, C1r and C1s that trigger activation upon binding to a microbial target...
  8. Wang H, Xue Z, Liu Z, Wang W, Wang F, Wang Y, et al. A novel C-type lectin from the sea cucumber Apostichopus japonicus (AjCTL-2) with preferential binding of d-galactose. Fish Shellfish Immunol. 2018;79:218-227 pubmed publisher
    ..It contained a canonical signal peptide at the N-terminus, a low density lipoprotein receptor class A (LDLa), a C1r/C1s/Uegf/bone morphogenetic protein 1 (CUB), and a CRD with two motifs Glu-Pro-Asn (EPN) and Trp-Asn-Asp (WND) in ..
  9. Guo J, Gao Y, Wang Y, Zou Y, Du Y, Luo C, et al. Investigation of C1-complex regions reveals new C1Q variants associated with protection from systemic lupus erythematosus, and affect its transcript abundance. Sci Rep. 2018;8:8048 pubmed publisher
    ..Furthermore, there are no reports concerning polymorphisms of C1S, C1R, and C1RL and whether they confer susceptibility to SLE...
  10. Gao Z, Li M, Ma J, Zhang S. An amphioxus gC1q protein binds human IgG and initiates the classical pathway: Implications for a C1q-mediated complement system in the basal chordate. Eur J Immunol. 2014;44:3680-95 pubmed publisher
    ..Moreover, rBjC1q can assemble to form the high molecular weight oligomers necessary for binding to proteases C1r/C1s and for complement activation, and binds human C1r/C1s/mannan-binding lectin-associated serine protease-2 as ..
  11. Tang M, Pelkey K, Ng D, Ivakine E, McBain C, Salter M, et al. Neto1 is an auxiliary subunit of native synaptic kainate receptors. J Neurosci. 2011;31:10009-18 pubmed publisher
    ..We previously identified the CUB (complement C1r/C1s, Uegf, Bmpl) domain protein, Neto1, as an NMDA receptor-associated polypeptide...
  12. Zhang S, Shaw Boden J, Banz Y, Bongoni A, Taddeo A, Spirig R, et al. Effects of C1 inhibitor on endothelial cell activation in a rat hind limb ischemia-reperfusion injury model. J Vasc Surg. 2018;68:209S-221S.e2 pubmed publisher
    ..At the end, muscle edema, tissue viability, and histologic features were assessed. Deposition of immunoglobulin M, C1r, C4d, and fibrin and expression of plasminogen activator inhibitor 1, heparan sulfate (HS), E-selectin, and ..
  13. Muia J, Zhu J, Gupta G, Haberichter S, Friedman K, Feys H, et al. Allosteric activation of ADAMTS13 by von Willebrand factor. Proc Natl Acad Sci U S A. 2014;111:18584-9 pubmed publisher
    ..Another seven T and two complement C1r/C1s, sea urchin epidermal growth factor, and bone morphogenetic protein (CUB) domains of uncertain function are C-..
  14. Nilojan J, Bathige S, Thulasitha W, Kwon H, Jung S, Kim M, et al. Transcriptional profiling, molecular cloning, and functional analysis of C1 inhibitor, the main regulator of the complement system in black rockfish, Sebastes schlegelii. Fish Shellfish Immunol. 2018;75:263-273 pubmed publisher
    ..For the first time, the anti-protease activity of the teleost C1inh against its natural substrates C1r and C1s was proved in this study...
  15. Chen L, Fischer R, Peng Y, Reeves E, McHugh K, Ternette N, et al. Critical role of endoplasmic reticulum aminopeptidase 1 in determining the length and sequence of peptides bound and presented by HLA-B27. Arthritis Rheumatol. 2014;66:284-94 pubmed publisher
    ..ERAP1-silenced and -competent HeLa.B27 and C1R.B27 cells were isotope-labeled, mixed, lysed, and then immunoprecipitated using W6/32 or ME1 antibodies...
  16. Yan W, Che L, Jiang J, Yang F, Duan Q, Song H, et al. Depletion of complement system immunity in patients with myocardial infarction. Mol Med Rep. 2016;14:5350-5356 pubmed publisher
    ..In the patients with AMI, the expression levels of genes encoding C1q?, C1q?, C1q?, C1r, Factor P, C5a (complement component), CR1, integrin ?M, integrin ?X, integrin ?2, C5aR, CRIg (complement receptors)..
  17. Chou C, Li Y, Chang C, Yang C, Li P, Jeng Y, et al. Prognostic Significance of CDCP1 Expression in Colorectal Cancer and Effect of Its Inhibition on Invasion and Migration. Ann Surg Oncol. 2015;22:4335-43 pubmed publisher
    To assess the correlations and functions of complement C1r/C1s, Uegf, Bmp1 domain-containing protein-1 (CDCP1) in identifying colorectal cancer (CRC) patients who are at high risk for metastasis...
  18. Uchio Yamada K, Tanaka M, Manabe N. C1r/C1s deficiency is insufficient to induce murine systemic lupus erythematosus. Genes Immun. 2019;20:121-130 pubmed publisher
    ..This deletion led to the absence of C1r/C1s and a low level of C1q in the circulation...
  19. Jin W, Ren L, Liu B, Zhang Q, Zhong W. Structural Features of Sulfated Glucuronomannan Oligosaccharides and Their Antioxidant Activity. Mar Drugs. 2018;16: pubmed publisher
    ..NMR techniques were used to reveal that the order of sulfation was Man-C6 > Man-C4 > Man-C1R > GlcA-C3 > Man-C3 > GlcA-C2...
  20. Bolayir H, Güneş H, Kivrak T, Sahin O, Akaslan D, Kurt R, et al. The role of SCUBE1 in the pathogenesis of no-reflow phenomenon presenting with ST segment elevation myocardial infarction. Anatol J Cardiol. 2017;18:122-127 pubmed publisher
    SCUBE1 [signal peptide-CUB (complement C1r/C1 s)-EGF (epidermal growth factor)-like domain-containing protein 1] might function as a novel platelet-endothelial adhesion molecule and play pathological roles in cardiovascular biology...
  21. Sönmez E, Turkdogan K, Karabacak M, Civelek C, Yilmaz C, Ozer O, et al. The diagnostic role of signal peptide-C1r/C1s, Uegf, and Bmp1-epidermal growth factor domain-containing protein 1 in non-ST-elevation acute coronary syndrome. Am J Emerg Med. 2015;33:21-4 pubmed publisher
    ..Signal peptide-C1r/C1s, Uegf, and Bmp1-epidermal growth factor domain-containing protein 1 (SCUBE1) is stored within the α granules ..
  22. Venkatraman Girija U, Furze C, Toth J, Schwaeble W, Mitchell D, Keeble A, et al. Engineering novel complement activity into a pulmonary surfactant protein. J Biol Chem. 2010;285:10546-52 pubmed publisher
    ..complement-specific serine proteases: MBLs and ficolins with MBL-associated serine protease-2 (MASP-2) and C1q with C1r and C1s...
  23. McDonald C, Ostini L, Bennett N, Subramaniam N, Hooper J, Velasco G, et al. Functional analysis of matriptase-2 mutations and domains: insights into the molecular basis of iron-refractory iron deficiency anemia. Am J Physiol Cell Physiol. 2015;308:C539-47 pubmed publisher
    ..PROT) domains from MT-2 with those from MT-1 resulted in limited cell surface localization, while the complement C1r/C1s, Uegf, Bmp1 (CUB) domain chimera retained localization at the cell surface...
  24. Leroy C, Shen Q, Strande V, Meyer R, McLaughlin M, Lezan E, et al. CUB-domain-containing protein 1 overexpression in solid cancers promotes cancer cell growth by activating Src family kinases. Oncogene. 2015;34:5593-8 pubmed publisher
    The transmembrane glycoprotein, CUB (complement C1r/C1s, Uegf, Bmp1) domain-containing protein 1 (CDCP1) is overexpressed in several cancer types and is a predictor of poor prognosis for patients on standard of care therapies...
  25. Almitairi J, Venkatraman Girija U, Furze C, Simpson Gray X, Badakshi F, Marshall J, et al. Structure of the C1r-C1s interaction of the C1 complex of complement activation. Proc Natl Acad Sci U S A. 2018;115:768-773 pubmed publisher
    ..It is formed from the recognition subcomponent C1q and a tetramer of proteases C1r2C1s2 as a Ca2+-dependent complex...
  26. Ugurlar D, Howes S, de Kreuk B, Koning R, de Jong R, Beurskens F, et al. Structures of C1-IgG1 provide insights into how danger pattern recognition activates complement. Science. 2018;359:794-797 pubmed publisher
    ..Upon antibody binding, the C1q arms condense, inducing rearrangements of the C1r2s2 proteases and tilting C1q's cone-shaped stalk...
  27. Goshima M, Sekiguchi R, Matsushita M, Nonaka M. The complement system of elasmobranches revealed by liver transcriptome analysis of a hammerhead shark, Sphyrna zygaena. Dev Comp Immunol. 2016;61:13-24 pubmed publisher
    ..Identified genes were; one C1qB, one C1r, one C1s, one MASP-1/-3, one MASP-2, two factor B/C2, one C3, three C4, one C5, one C6, one C7, one C8A, three C8B, ..
  28. Sato T, Iyama S, Murase K, Kamihara Y, Ono K, Kikuchi S, et al. Novel missense mutation in the TMPRSS6 gene in a Japanese female with iron-refractory iron deficiency anemia. Int J Hematol. 2011;94:101-103 pubmed publisher
    ..paper, we describe the first case of a Japanese female with IRIDA, who carried a novel mutation (K253E) in the CUB (complement factor C1r/C1s, urchin embryonic growth factor and bone morphogenetic protein 1) domain of the TMPRSS6 gene.
  29. Kobayashi H, Albarracín L, Sato N, Kanmani P, Kober A, Ikeda Ohtsubo W, et al. Modulation of porcine intestinal epitheliocytes immunetranscriptome response by Lactobacillus jensenii TL2937. Benef Microbes. 2016;7:769-782 pubmed
    ..The decrease in the expression of chemokines (CCL8, CXCL5, CXCL9, CXCL10, and CXCL11), complement (C1R, C1S, C3, and CFB), and coagulation factors (F3) by L...
  30. Chen L, Shi H, Yuan J, Bowness P. Position 97 of HLA-B, a residue implicated in pathogenesis of ankylosing spondylitis, plays a key role in cell surface free heavy chain expression. Ann Rheum Dis. 2017;76:593-601 pubmed publisher
    ..Flow cytometry was used to measure surface expression of HLA-B*27 in C1R/HeLa cells expressing HLA-B*27 (N97) and six mutants at P97 (N97T, N97S, N97V, N97R, N97W and N97D)...
  31. Park H, You G, Yang K, Kim J, An S, Song J, et al. Role of AKT and ERK pathways in controlling sensitivity to ionizing radiation and adaptive response induced by low-dose radiation in human immune cells. Eur J Cell Biol. 2015;94:653-60 pubmed publisher
    ..However, IM-9 and C1R-sB7 cells were very sensitive to high-dose radiation-induced cell death, whereas Jurkat and JM1 cells showed ..
  32. Zhang Z, Ciccia F, Zeng F, Guggino G, Yee K, Abdullah H, et al. Brief Report: Functional Interaction of Endoplasmic Reticulum Aminopeptidase 2 and HLA-B27 Activates the Unfolded Protein Response. Arthritis Rheumatol. 2017;69:1009-1015 pubmed publisher
    ..ERAP2-suppressed, stable B27-expressing C1R cells (C1R-B27) were tested for the expression levels of pB27 and FHCs, as well as for markers of the unfolded ..
  33. Mortensen S, Sander B, Jensen R, Pedersen J, Golas M, Jensenius J, et al. Structure and activation of C1, the complex initiating the classical pathway of the complement cascade. Proc Natl Acad Sci U S A. 2017;114:986-991 pubmed publisher
    ..binding of the 774-kDa C1 complex, consisting of the recognition molecule C1q and the tetrameric protease complex C1r2s2, to a variety of activators presenting specific molecular patterns such as IgG- and IgM-..
  34. Wang Z, Wang Z, Zhou Z, Ren Y. Crucial genes associated with diabetic nephropathy explored by microarray analysis. BMC Nephrol. 2016;17:128 pubmed publisher
    ..g. ACTN4, CTNNA1 and ITGB5), as well as complement and coagulation cascades (e.g. C1R and C1S). Furthermore, a series of miRNAs and TFs modulating DEGs were identified...
  35. Godahewa G, Bathige S, Herath H, Noh J, Lee J. Characterization of rock bream (Oplegnathus fasciatus) complement components C1r and C1s in terms of molecular aspects, genomic modulation, and immune responsive transcriptional profiles following bacterial and viral pathogen exposure. Fish Shellfish Immunol. 2015;46:656-68 pubmed publisher
    The complement components C1r and C1s play a crucial role in innate immunity via activation of the classical complement cascade system. As initiators of the pathogen-induced signaling cascade, C1r and C1s modulate innate immunity...
  36. Lee S, Kim K, Joo K, Kim J. Angiogenin ameliorates corneal opacity and neovascularization via regulating immune response in corneal fibroblasts. BMC Ophthalmol. 2016;16:57 pubmed publisher
    ..the mRNA levels of IL-1?, IL-6, IL-8, TNF-? receptor (TNFR)1, 2, TLR4, MYD88, and complement components except for C1r and C1s and elevated the mRNA expression of IL-4 and IL-10...
  37. Ong O, Young L, Old J. Sequences and expression of pathway-specific complement components in developing red-tailed phascogale (Phascogale calura). Dev Comp Immunol. 2016;65:314-320 pubmed publisher
    ..We report partial cDNA sequences for two complement-activating proteins, C3, C1r, CFP and MASP2, in liver tissues from red-tailed phascogale (Phascogale calura)...
  38. Turedi S, Tatli O, Alver A, Karaguzel E, Karaca Y, Turkmen S, et al. The Diagnostic Value of Plasma SCUBE1, a Novel Biomarker of Platelet Activation, in Testicular Torsion: A Randomized, Controlled, Experimental Study. Urology. 2015;86:516-20 pubmed publisher
    To investigate the potential diagnostic value of plasma signal peptide, CUB (complement proteins C1r/C1s, Uegf, Bmp1) domain, epidermal growth factor (EGF)-like 1 (SCUBE1) protein in experimentally induced testicular torsion (TT)...
  39. Kaplan A, Joseph K. Complement, Kinins, and Hereditary Angioedema: Mechanisms of Plasma Instability when C1 Inhibitor is Absent. Clin Rev Allergy Immunol. 2016;51:207-15 pubmed publisher
    ..These phenomena are due to the properties of the C1r subcomponent of C1, factor XII, and the bimolecular complex of prekallikrein with high molecular weight kininogen (..
  40. Kawahara R, Bollinger J, Rivera C, Ribeiro A, Brandão T, Paes Leme A, et al. A targeted proteomic strategy for the measurement of oral cancer candidate biomarkers in human saliva. Proteomics. 2016;16:159-73 pubmed publisher
    ..Using Skyline software package, we demonstrated a statistically significant higher abundance of the C1R, LCN2, SLPI, FAM49B, TAGLN2, CFB, C3, C4B, LRG1, SERPINA1 candidate biomarkers in the saliva of OSCC patients...
  41. Petitbarat M, Durigutto P, Macor P, Bulla R, Palmioli A, Bernardi A, et al. Critical Role and Therapeutic Control of the Lectin Pathway of Complement Activation in an Abortion-Prone Mouse Mating. J Immunol. 2015;195:5602-7 pubmed publisher
    ..components localized at the implantation sites of CBA/J × DBA/2, we found C4 and C3, but we failed to reveal C1r. These data suggest that complement is activated through the lectin pathway and proceeds to completion of the ..
  42. Lepedda A, Lobina O, Rocchiccioli S, Nieddu G, Ucciferri N, De Muro P, et al. Identification of differentially expressed plasma proteins in atherosclerotic patients with type 2 diabetes. J Diabetes Complications. 2016;30:880-6 pubmed publisher
    ..Among them, there were fibrinogen beta and gamma chains, complement C1r, C3 and C4-B subcomponents, alpha-1-antitrypsin (AAT), vitronectin and CD5 antigen-like...
  43. Schaer C, Owczarek C, Deuel J, Schauer S, Baek J, Yalamanoglu A, et al. Phenotype-specific recombinant haptoglobin polymers co-expressed with C1r-like protein as optimized hemoglobin-binding therapeutics. BMC Biotechnol. 2018;18:15 pubmed publisher
    ..ProHp is proteolytically cleaved by complement C1r subcomponent-like protein (C1r-LP) in the endoplasmic reticulum. Two main allelic Hp variants, HP1 and HP2 exist...
  44. Abdullah H, Zhang Z, Yee K, Haroon N. KIR3DL1 interaction with HLA-B27 is altered by ankylosing spondylitis associated ERAP1 and enhanced by MHC class I cross-linking. Discov Med. 2015;20:79-89 pubmed
    ..Secondarily we observed that KIR3DL1 binding to C1R-B27 APCs is enhanced by blocking pHLA, but not FHC, raising the possibility that antibody mediated HLA-B27 cross-..
  45. Gautam A, Panse Y, Ghosh P, Reza M, Mullick J, Sahu A. Mutational analysis of Kaposica reveals that bridging of MG2 and CUB domains of target protein is crucial for the cofactor activity of RCA proteins. Proc Natl Acad Sci U S A. 2015;112:12794-9 pubmed publisher
    ..Furthermore, the model suggested that the C3b-interacting residues bridge the CUB (complement C1r-C1s, Uegf, Bmp1) and MG2 (macroglobulin-2) domains of C3b...
  46. Xavier S, Sahu R, Landes S, Yu J, Taylor R, Ayyadevara S, et al. Pericytes and immune cells contribute to complement activation in tubulointerstitial fibrosis. Am J Physiol Renal Physiol. 2017;312:F516-F532 pubmed publisher
    ..and flow cytometry analysis performed in whole kidney tissue confirmed increased expression of C1q, C1r, and C1s as well as complement activation, which is measured as increased synthesis of C3 fragments predominantly ..
  47. Truedsson L. Classical pathway deficiencies - A short analytical review. Mol Immunol. 2015;68:14-9 pubmed publisher
    ..Deficiencies of each of the components (C1q, C1s, C1r, C4 and C2) imply increased susceptibility to bacterial infections...
  48. Wright H, Arulmoli J, Motazedi M, Nelson L, Heinemann F, Flanagan L, et al. CDCP1 cleavage is necessary for homodimerization-induced migration of triple-negative breast cancer. Oncogene. 2016;35:4762-72 pubmed publisher
    ..TNBCs express high levels of the transmembrane glycoprotein, complement C1r/C1s, Uegf, Bmp1 (CUB)-domain containing protein 1 (CDCP1), which has been correlated with the aggressiveness and ..
  49. Chen M, Wu J, Shi S, Chen Y, Wang H, Fan H, et al. Structure analysis of a heteropolysaccharide from Taraxacum mongolicum Hand.-Mazz. and anticomplementary activity of its sulfated derivatives. Carbohydr Polym. 2016;152:241-252 pubmed publisher
    ..42±0.22?g/mL). Mechanism studies indicated that Sul-DPSW-A inhibited complement activation by blocking C1q, C1r, C1s, and C9, but not C2, C3, C4, and C5. In addition, Sul-DPSW-A displayed limited anticoagulant effects...
  50. Luedecking Zimmer E, DeKosky S, Nebes R, Kamboh M. Association of the 3' UTR transcription factor LBP-1c/CP2/LSF polymorphism with late-onset Alzheimer's disease. Am J Med Genet B Neuropsychiatr Genet. 2003;117B:114-7 pubmed
    ..The genes included complement component 1R (C1R), vitamin D receptor (VDR), scavenger-receptor B1 (SR-B1), low-density lipoprotein receptor related protein 1 (LRP1)..
  51. Arlaud G, Willis A, Gagnon J. Complete amino acid sequence of the A chain of human complement-classical-pathway enzyme C1r. Biochem J. 1987;241:711-20 pubmed
    The amino acid sequence of human C1r A chain was determined, from sequence analysis performed on fragments obtained from C1r autolytic cleavage, cleavage of methionyl bonds, tryptic cleavages at arginine and lysine residues, and ..
  52. Di Marco M, Schuster H, Backert L, Ghosh M, Rammensee H, Stevanovic S. Unveiling the Peptide Motifs of HLA-C and HLA-G from Naturally Presented Peptides and Generation of Binding Prediction Matrices. J Immunol. 2017;199:2639-2651 pubmed publisher
    ..most frequently expressed HLA-C alleles as well as HLA-E*01:01 and HLA-G*01:01 were transfected into lymphoblastoid C1R cells expressing low endogenous HLA...
  53. Nöthen M, Dewald G. A common amino acid polymorphism in complement component C1R. Hum Mol Genet. 1994;3:217 pubmed
  54. Pflieger D, Przybylski C, Gonnet F, Le Caer J, Lunardi T, Arlaud G, et al. Analysis of human C1q by combined bottom-up and top-down mass spectrometry: detailed mapping of post-translational modifications and insights into the C1r/C1s binding sites. Mol Cell Proteomics. 2010;9:593-610 pubmed publisher
    ..They are thus fully available and in an appropriate position to interact with the C1r and C1s protease partners of C1q and are therefore likely to play an essential role in C1 assembly.
  55. Watanabe A, Satoh K, Maniwa T, Matsumoto K. Proteomic analysis for the identification of serum diagnostic markers for joint hypermobility syndrome. Int J Mol Med. 2016;37:461-7 pubmed publisher
    ..05, patient vs. control). Of the 6 proteins, proteins involved in the complement system including complement C1r subcomponent (C1R), vitronectin (VTN), complement component C9 (C9), and C4b-binding protein alpha chain (C4BPA) ..
  56. Yammani R, Seetharam S, Seetharam B. Cubilin and megalin expression and their interaction in the rat intestine: effect of thyroidectomy. Am J Physiol Endocrinol Metab. 2001;281:E900-7 pubmed
    ..and a contiguous stretch (representing nearly 88% of its mass) of 27 CUB (initially found in complement components C1r/C1s, Uegf, and bone morphogenic protein-1) domains...
  57. Brier S, Pflieger D, Le Mignon M, Bally I, Gaboriaud C, Arlaud G, et al. Mapping surface accessibility of the C1r/C1s tetramer by chemical modification and mass spectrometry provides new insights into assembly of the human C1 complex. J Biol Chem. 2010;285:32251-63 pubmed publisher
    ..association of a recognition protein C1q with a Ca(2+)-dependent tetramer comprising two copies of the proteases C1r and C1s...
  58. Láng A, Major B, Szilágyi K, Gáspári Z, Gal P, Zavodszky P, et al. Interaction between separated consecutive complement control modules of human C1r: implications for dimerization of the full-length protease. FEBS Lett. 2010;584:4565-9 pubmed publisher
    ..lacking experimental evidence for intermolecular interactions between the CCP1 and CCP2 modules of the human C1r serine protease (SP)...
  59. Budayova Spano M, Lacroix M, Thielens N, Arlaud G, Fontecilla Camps J, Gaboriaud C. The crystal structure of the zymogen catalytic domain of complement protease C1r reveals that a disruptive mechanical stress is required to trigger activation of the C1 complex. EMBO J. 2002;21:231-9 pubmed
    b>C1r is the modular serine protease (SP) that mediates autolytic activation of C1, the macromolecular complex that triggers the classical pathway of complement...
  60. Journet A, Tosi M. Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r. Biochem J. 1986;240:783-7 pubmed
    The sequencing of human liver cDNA clones encoding the entire C1r precursor protein has confirmed the previously determined peptide sequence and has shown that there is a leader peptide which is 17 amino acids long...
  61. Gasque P, Ischenko A, Legoedec J, Mauger C, Schouft M, Fontaine M. Expression of the complement classical pathway by human glioma in culture. A model for complement expression by nerve cells. J Biol Chem. 1993;268:25068-74 pubmed
    In this paper we demonstrate the synthesis of the components of the classical complement pathway, namely C1q, C1r, C1s, C1-Inh, C2, C4, and C5, by human glioma cell lines (U118MG, T193, and T98G)...
  62. Baranzini S, Elfstrom C, Chang S, Butunoi C, Murray R, Higuchi R, et al. Transcriptional analysis of multiple sclerosis brain lesions reveals a complex pattern of cytokine expression. J Immunol. 2000;165:6576-82 pubmed
    ..Overall, our data suggest a complex regulation of the inflammatory response in human autoimmune demyelination. ..
  63. Kapferer Seebacher I, Pepin M, Werner R, Aitman T, Nordgren A, Stoiber H, et al. Periodontal Ehlers-Danlos Syndrome Is Caused by Mutations in C1R and C1S, which Encode Subcomponents C1r and C1s of Complement. Am J Hum Genet. 2016;99:1005-1014 pubmed publisher
    ..In 17 of these families, we identified heterozygous missense or in-frame insertion/deletion mutations in C1R (15 families) or C1S (2 families), contiguous genes in the mapped locus that encode subunits C1r and C1s of the ..
  64. Kardos J, Harmat V, Palló A, Barabas O, Szilágyi K, Gráf L, et al. Revisiting the mechanism of the autoactivation of the complement protease C1r in the C1 complex: structure of the active catalytic region of C1r. Mol Immunol. 2008;45:1752-60 pubmed
    b>C1r is a modular serine protease which is the autoactivating component of the C1 complex of the classical pathway of the complement system. We have determined the first crystal structure of the entire active catalytic region of human C1r...
  65. Wu C, Xu G, Tsai S, Freed W, Lee C. Transcriptional profiles of type 2 diabetes in human skeletal muscle reveal insulin resistance, metabolic defects, apoptosis, and molecular signatures of immune activation in response to infections. Biochem Biophys Res Commun. 2017;482:282-288 pubmed publisher
    ..include immune-related pathways suggestive of a response to various infectious diseases (C2, CFB, C4A, C4B, C1S, C1R, C3, HLA-DRA, HLA-DMA, HLA-DOA, and HLA-DPB1)...
  66. Arlaud G, Gagnon J. Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r. Biochemistry. 1983;22:1758-64 pubmed
    ..Two carbohydrate moieties are attached to the polypeptide chain, both via asparagine residues at positions 51 and 118. ..
  67. Major B, Kardos J, Kékesi K, Lorincz Z, Zavodszky P, Gal P. Calcium-dependent conformational flexibility of a CUB domain controls activation of the complement serine protease C1r. J Biol Chem. 2010;285:11863-9 pubmed publisher
    ..of the complement system, is a Ca(2+)-dependent heteropentamer complex of C1q and two modular serine proteases, C1r and C1s. Current functional models assume significant flexibility of the subcomponents...
  68. Hovingh E, van den Broek B, Kuipers B, Pinelli E, Rooijakkers S, Jongerius I. Acquisition of C1 inhibitor by Bordetella pertussis virulence associated gene 8 results in C2 and C4 consumption away from the bacterial surface. PLoS Pathog. 2017;13:e1006531 pubmed publisher
    ..We reveal that Vag8 binding to human C1-inhibitor (C1-inh) interferes with the binding of C1-inh to C1s, C1r and MASP-2, resulting in the release of active proteases that subsequently cleave C2 and C4 away from the bacterial ..
  69. Leytus S, Kurachi K, Sakariassen K, Davie E. Nucleotide sequence of the cDNA coding for human complement C1r. Biochemistry. 1986;25:4855-63 pubmed
    b>C1r is a zymogen of a serine protease that is involved in the activation of the first component of the classical pathway of the complement system...
  70. Lee S, Wallace S, Barone R, Blum L, Chase P. Familial deficiency of two subunits of the first component of complement. C1r and C1s associated with a lupus erythematosus-like disease. Arthritis Rheum. 1978;21:958-67 pubmed
    Complete absence of C1r and almost complete absence of C1s were found in 4 of 8 living siblings...
  71. Demirkaya E, Zhou Q, Smith C, Ombrello M, Deuitch N, Tsai W, et al. Brief Report: Deficiency of Complement 1r Subcomponent in Early-Onset Systemic Lupus Erythematosus: The Role of Disease-Modifying Alleles in a Monogenic Disease. Arthritis Rheumatol. 2017;69:1832-1839 pubmed publisher
    ..We identified a novel, homozygous, loss-of-function mutation (p.Pro445Leufs*11) in the C1R gene...
  72. Busby T, Ingham K. NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with C1q. Biochemistry. 1990;29:4613-8 pubmed
    ..of human complement, involves interactions between various domains of each of its three subcomponents, C1q, C1r, and C1s...
  73. Cai Y, Wee S, Chen J, Teo B, Ng Y, Leong K, et al. Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation. J Immunol. 2017;199:3981-3990 pubmed publisher
    ..a recent finding that, in dead cells, nucleoli were targeted by C1q and two nucleolar autoantigens were degraded by C1r/C1s proteases, we considered that C1 could help protect against antinuclear autoimmunity by broadly degrading ..
  74. Budayova Spano M, Grabarse W, Thielens N, Hillen H, Lacroix M, Schmidt M, et al. Monomeric structures of the zymogen and active catalytic domain of complement protease c1r: further insights into the c1 activation mechanism. Structure. 2002;10:1509-19 pubmed
    b>C1r is the serine protease (SP) that mediates autoactivation of C1, the complex that triggers the classical complement pathway...
  75. Gregory L, Thielens N, Arlaud G, Fontecilla Camps J, Gaboriaud C. X-ray structure of the Ca2+-binding interaction domain of C1s. Insights into the assembly of the C1 complex of complement. J Biol Chem. 2003;278:32157-64 pubmed
    C1, the complex that triggers the classical pathway of complement, is assembled from two modular proteases C1r and C1s and a recognition protein C1q...
  76. Thielens N, Enrie K, Lacroix M, Jaquinod M, Hernandez J, Esser A, et al. 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. J Biol Chem. 1999;274:9149-59 pubmed
    The Ca2+-dependent interaction between complement serine proteases C1r and C1s is mediated by their alpha regions, encompassing the major part of their N-terminal CUB-EGF-CUB (where EGF is epidermal growth factor) module array...
  77. Thiel S, Petersen S, Vorup Jensen T, Matsushita M, Fujita T, Stover C, et al. Interaction of C1q and mannan-binding lectin (MBL) with C1r, C1s, MBL-associated serine proteases 1 and 2, and the MBL-associated protein MAp19. J Immunol. 2000;165:878-87 pubmed
    Mannan-binding lectin (MBL) and C1q activate the complement cascade via attached serine proteases. The proteases C1r and C1s were initially discovered in a complex with C1q, whereas the MBL-associated serine proteases 1 and 2 (MASP-1 and ..
  78. Zeis T, Enz L, Schaeren Wiemers N. The immunomodulatory oligodendrocyte. Brain Res. 2016;1641:139-148 pubmed publisher
    ..g. CD9, CD81), complement and complement receptor molecules (e.g. C1s, C2 and C3, C1R), complement regulatory molecules (e.g. CD46, CD55, CD59), tetraspanins (e.g...
  79. Sanfilippo C, Cambria D, Longo A, Palumbo M, Avola R, Pinzone M, et al. SERPING1 mRNA overexpression in monocytes from HIV+ patients. Inflamm Res. 2017;66:1107-1116 pubmed publisher
    ..SERPING1 is a protease inhibitor that disables C1r/C1s in the C1 complex of the classical complement pathway...
  80. Nilojan J, Bathige S, Kugapreethan R, Yang H, Kim M, Nam B, et al. Molecular features and the transcriptional and functional delineation of complement system activators C1r and C1s from Sebastes schlegelii. Dev Comp Immunol. 2018;81:279-290 pubmed publisher
    b>C1r and C1s are serine proteases responsible for activating the classical complement pathway to initiate the complement cascade, which plays a crucial role in eliminating invading pathogenic microbes...
  81. Pelloux S, Thielens N, Hudry Clergeon G, Petillot Y, Filhol O, Arlaud G. Identification of a cryptic protein kinase CK2 phosphorylation site in human complement protease Clr, and its use to probe intramolecular interaction. FEBS Lett. 1996;386:15-20 pubmed
    Treatment of human (activated)C1r by CK2 resulted in the incorporation of [32P]phosphate into the N-terminal alpha region of its non-catalytic A chain...
  82. Wang H, Li N, Zhu C, Shi S, Jin H, Wang S. Anti-complementary activity of two homogeneous polysaccharides from Eclipta prostrata. Biochem Biophys Res Commun. 2017;493:887-893 pubmed publisher
    ..Preliminary mechanism study indicated that both EAP20-1 and EAP20-2 inhibited the activation of the complement system by interacting with C1q, C1r, C1s, C2, C4, C5, C7, and C9 components.
  83. Bersch B, Hernandez J, Marion D, Arlaud G. Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, an atypical member of the EGF family. Biochemistry. 1998;37:1204-14 pubmed
    The calcium-dependent interaction between C1r and C1s, the two homologous serine proteases of the first component of human complement C1, is mediated by their N-terminal regions...
  84. Cai Y, Teo B, Yeo J, Lu J. C1q protein binds to the apoptotic nucleolus and causes C1 protease degradation of nucleolar proteins. J Biol Chem. 2015;290:22570-80 pubmed publisher
    ..In vivo, C1q exists as the C1 complex (C1qC1r2C1s2), and C1q binding to ligands activates the C1r/C1s proteases...
  85. Nguyen V, Tosi M, Gross M, Cohen Haguenauer O, Jegou Foubert C, de Tand M, et al. Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13. Hum Genet. 1988;78:363-8 pubmed
    b>C1r and C1s are distinct, but structurally and functionally similar, serine protease zymogens responsible for the enzymatic activity of the first component of complement (C1)...
  86. Láng A, Szilágyi K, Major B, Gal P, Zavodszky P, Perczel A. Intermodule cooperativity in the structure and dynamics of consecutive complement control modules in human C1r: structural biology. FEBS J. 2010;277:3986-98 pubmed publisher
    The modular C1r protein is the first protease activated in the classical complement pathway, a key component of innate immunity...
  87. Arlaud G, Van Dorsselaer A, Bell A, Mancini M, Aude C, Gagnon J. Identification of erythro-beta-hydroxyasparagine in the EGF-like domain of human C1r. FEBS Lett. 1987;222:129-34 pubmed
    Previous studies [(1987) Biochem. J. 241, 711-720] have shown that position 150 of human C1r is occupied by a modified amino acid that, after acid hydrolysis, yields erythro-beta-hydroxyaspartic acid...
  88. Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens N, et al. 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. 1997;36:6270-82 pubmed
    b>C1r is the modular serine protease responsible for autocatalytic activation of C1, the first component of the complement classical pathway...
  89. Pang S, Wijeyewickrema L, Hor L, Tan S, Lameignere E, Conway E, et al. The Structural Basis for Complement Inhibition by Gigastasin, a Protease Inhibitor from the Giant Amazon Leech. J Immunol. 2017;199:3883-3891 pubmed publisher
    ..The classical and lectin pathways are initiated by the C1r/C1s (classical) and MASP-1/MASP-2 (lectin) proteases...
  90. Wijeyewickrema L, Yongqing T, Tran T, Thompson P, Viljoen J, Coetzer T, et al. Molecular determinants of the substrate specificity of the complement-initiating protease, C1r. J Biol Chem. 2013;288:15571-80 pubmed publisher
    The serine protease, C1r, initiates activation of the classical pathway of complement, which is a crucial innate defense mechanism against pathogens and altered-self cells...
  91. Lyons L, Kamboh M, Ferrell R. Genetic studies of low-abundance human plasma proteins. XI. Linkage analysis and population genetics of the C1S subcomponent of the first complement component. Complement Inflamm. 1989;6:81-7 pubmed
    Although subcomponents C1r and C1s of the first complement component, C1, have been established to be in the same linkage group as the proline-rich protein gene cluster on chromosome 12p13...
  92. Luo C, Thielens N, Gagnon J, Gal P, Sarvari M, Tseng Y, et al. 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. 1992;31:4254-62 pubmed
    ..mutated protein displays good hemolytic activity when reassembled with C1q and either human serum or recombinant C1r, demonstrating that these posttranslational modifications are not critical for any of the multiple interactions ..