The C3b/C4b receptor is recognized by the Knops, McCoy, Swain-langley, and York blood group antisera.

Nickells, Michael; Moulds, John J.; Brown, Marion F.; Atkinson, John P.

doi:10.1084/jem.173.5.1159

Cited by 108 publications

(73 citation statements)

References 17 publications

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“…Knops-McCoy blood group antiserum, which is specific for CR1, served as a positive control. 32 Positive patient samples were confirmed specific for sCR1 by preincubating with excess sCR1 and rerunning the assay.…”

Section: Immunogenicity Assaysmentioning

confidence: 99%

Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy

Zhang

Nester

Holanda

et al. 2013

Journal of the American Society of Nephrology

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Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are widely recognized subtypes of C3 glomerulopathy. These ultra-rare renal diseases are characterized by fluid-phase dysregulation of the alternative complement pathway that leads to deposition of complement proteins in the renal glomerulus. Disease triggers are unknown and because targeted treatments are lacking, progress to end stage renal failure is a common final outcome. We studied soluble CR1, a potent regulator of complement activity, to test whether it restores complement regulation in C3 glomerulopathy. In vitro studies using sera from patients with DDD showed that soluble CR1 prevents dysregulation of the alternative pathway C3 convertase, even in the presence of C3 nephritic factors. In mice deficient in complement factor H and transgenic for human CR1, soluble CR1 therapy stopped alternative pathway activation, resulting in normalization of serum C3 levels and clearance of iC3b from glomerular basement membranes. Shortterm use of soluble CR1 in a pediatric patient with end stage renal failure demonstrated its safety and ability to normalize activity of the terminal complement pathway. Overall, these data indicate that soluble CR1 re-establishes regulation of the alternative complement pathway and provide support for a limited trial to evaluate soluble CR1 as a treatment for DDD and C3GN. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are two widely recognized subtypes of C3 glomerulopathy (C3G). 1,2 These ultra-rare renal diseases are caused by fluid-phase dysregulation of the C3 convertase of the alternative pathway (AP) of complement, with variable concomitant dysregulation of the C5 convertase. Consistent with complement-mediated disease acting through the AP, C3G is strongly positive for C3 and notably negative for Igs by immunofluorescence microscopy. 2 Electron microscopy distinguishes DDD from C3GN, with the former characterized by pathognomonic electron-dense transformation of the lamina densa of the glomerular basement membrane (GBM). 3 In C3GN, the electron microscopy deposits are lighter in color, and are more often mesangial and/or subendothelial, intramembranous, and subepithelial in location. 4 In both diseases, mass spectroscopy of laser dissected glomeruli is highly enriched for proteins of the AP and terminal complement cascade. 4,5

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Section: Immunogenicity Assaysmentioning

confidence: 99%

Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy

Zhang

Nester

Holanda

et al. 2013

Journal of the American Society of Nephrology

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“…[6][7][8] In 1991, two groups independently identified the Knops blood group system on CR1. 9,10 It has been shown to be independent from the other known CR1 polymorphisms, ie, molecular weight or red cell expression. The Knops blood group system began with the description of anti-Knops (Kn a ), in a transfused Caucasian woman.…”

Section: Introductionmentioning

confidence: 99%

Identification of complement receptor one (CR1) polymorphisms in West Africa

Moulds¹,

Kassambara

Middleton³

et al. 2000

Genes Immun

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Complement receptor one (CR1) is a ligand for the rosetting of Plasmodium falciparum infected red cells with uninfected cells. Since CR1 exhibits three known polymorphisms, we studied European-Americans (n = 112) and African-Americans (n = 330) and Malians (n = 158) to determine if genetic differences existed in an area endemic for malaria that could offer a survival advantage. The frequencies of Knops blood group phenotypes McC(b+) and Sl(a−) were greatly increased in Africans vs Europeans. Although the frequency of McC(b+) was similar between Africans from the USA or Mali, the Sl(a−) phenotype was significantly higher in Mali (39% vs 65%, respectively). There was an increased frequency of the largest size (250 kD) of CR1 in Mali, but this did not differ significantly from the USA (P = 0.09). Both cohorts of Africans had higher expression of red cell CR1 than European-Americans but this showed little difference between the USA and Mali groups. Thus, the most important CR1 polymorphism relevant to rosetting of malaria infected cells appears to be the Knops blood group. Genes and Immunity (2000) 1, 325-329.

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“…Another European study, which used only RFLP analysis, also found an increase of the CR1-B allele [17]. However, the latter was performed before the Knops-CR1 blood group was found [25], and it is unknown whether the RFLP actually correlates with one of the blood group specificities and not with CR1 size.…”

Section: Discussionmentioning

confidence: 99%

Structural polymorphisms of complement receptor 1 (CR1) in systemic lupus erythematosus (SLE) patients and normal controls of three ethnic groups

Moulds¹,

Reveille²,

Arnett³

1996

Clinical and Experimental Immunology

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SUMMARYCR1 exhibits a molecular weight polymorphism and variability in the number of C3b-binding sites. Because this may affect immune complex clearance, we used erythrocytes to investigate the CR1 size polymorphism in SLE patients from three ethnic groups. The CR1-C allele was found more frequently in African-Americans, but the frequency did not differ between controls (10%, n 63) and SLE patients (9%, n 79). A 160-kD band similar to CR1-C was noted in a number of patients and was shown to be a proteolytic cleavage fragment. The study shows that the smallest form of CR1, i.e. CR1-C, is not a genetic risk factor for SLE and that the frequencies of the CR1 structural alleles do not differ from race-matched healthy controls.

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The C3b/C4b receptor is recognized by the Knops, McCoy, Swain-langley, and York blood group antisera.

Cited by 108 publications

References 17 publications

Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy

Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy

Identification of complement receptor one (CR1) polymorphisms in West Africa

Structural polymorphisms of complement receptor 1 (CR1) in systemic lupus erythematosus (SLE) patients and normal controls of three ethnic groups

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