ArticleJournal of the American Society of Nephrology : JASN2013
Soluble CR1 therapy improves complement regulation in C3 glomerulopathy.
Article in Journal of the American Society of Nephrology : JASN, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
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Who cites it
46 citing papers in PubMed.
- Complement dysregulation associated with a genetic variant in factor H-related protein 5 in atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2024Article
- Complements and Their Role in Systemic Disorders.Cureus · 2024Review
- Protein therapeutics and their lessons: Expect the unexpected when inhibiting the multi-protein cascade of the complement system.Immunological reviews · 2023Review
- Increasing the efficacy and safety of a human complement inhibitor for treating post-transplant cardiac ischemia reperfusion injury by targeting to a graft-specific neoepitope.The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2021Article
- Associations between the Complement System and Choroidal Neovascularization in Wet Age-Related Macular Degeneration.International journal of molecular sciences · 2020Review
- Article
- COVID-19 cytokine storm: The anger of inflammation.Cytokine · 2020Review
- Nephritic Factors: An Overview of Classification, Diagnostic Tools and Clinical Associations.Frontiers in immunology · 2019Review
- Emerging immunotherapies for autoimmune kidney disease.Human vaccines & immunotherapeutics · 2019Review
- Altered Expression of Complement Regulatory Proteins CD35, CD46, CD55, and CD59 on Leukocyte Subsets in Individuals Suffering From Coronary Artery Disease.Frontiers in immunology · 2019Article
- Complement-mediated renal diseases after kidney transplantation - current diagnostic and therapeutic options inWorld journal of transplantation · 2018Review
- Thrombotic microangiopathy after renal transplantation: Current insights inWorld journal of transplantation · 2018Review
- The MFHR1 Fusion Protein Is a Novel Synthetic Multitarget Complement Inhibitor with Therapeutic Potential.Journal of the American Society of Nephrology : JASN · 2018Article
- The complement system in hypertension and renal damage in the Dahl SS rat.Physiological reports · 2018Article
- The renaissance of complement therapeutics.Nature reviews. Nephrology · 2018Review
- Diseases of complement dysregulation-an overview.Seminars in immunopathology · 2018Review
- Complement in ANCA-associated vasculitis: mechanisms and implications for management.Nature reviews. Nephrology · 2017Review
- Complement C3-Targeted Therapy: Replacing Long-Held Assertions with Evidence-Based Discovery.Trends in immunology · 2017Article
- Dysregulation of mCD46 and sCD46 contribute to the pathogenesis of bullous pemphigoid.Scientific reports · 2017Article
- Complement in Non-Antibody-Mediated Kidney Diseases.Frontiers in medicine · 2017Review
Corrections and comments
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Authors and funding
10 authors.
Funding
Abstract
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. Short-term 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.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.