Tipping the balance: intricate roles of the complement system in disease and therapy

Chemical synthesis and characterisation of the complement C5 inhibitory peptide zilucoplan

(2021) Declan M. Gorman et al.   AMINO ACIDS

Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease

(2021) Ahmet Ozen et al.   NATURE IMMUNOLOGY

Role of the C5a-C5a receptor axis in the inflammatory responses of the lungs after experimental polytrauma and hemorrhagic shock

(2021) Shinjini Chakraborty et al.   Scientific Reports

Avacopan for the Treatment of ANCA-Associated Vasculitis

(2021) David R.W. Jayne et al.   NEW ENGLAND JOURNAL OF MEDICINE

Deregulation of Factor H by Factor H-Related Protein 1 Depends on Sialylation of Host Surfaces

(2021) Arthur Dopler et al.   Frontiers in Immunology

Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria

(2021) Peter Hillmen et al.   NEW ENGLAND JOURNAL OF MEDICINE

Sutimlimab in Cold Agglutinin Disease

(2021) Alexander Röth et al.   NEW ENGLAND JOURNAL OF MEDICINE

Gain-of-function factor H–related 5 protein impairs glomerular complement regulation resulting in kidney damage

(2021) Talat H. Malik et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Sez6 Family Inhibits Complement by Facilitating Factor I Cleavage of C3b and Accelerating the Decay of C3 Convertases

(2021) Wen Q. Qiu et al.   Frontiers in Immunology

Increased complement activation is a distinctive feature of severe SARS-CoV-2 infection

(2021) Lina Ma et al.   Science Immunology

Common haplotypes at the CFH locus and low-frequency variants in CFHR2 and CFHR5 associate with systemic FHR concentrations and age-related macular degeneration

(2021) Laura Lorés-Motta et al.   AMERICAN JOURNAL OF HUMAN GENETICS

From discovery to approval: A brief history of the compstatin family of complement C3 inhibitors

(2021) Dimitrios C. Mastellos et al.   CLINICAL IMMUNOLOGY

Fc Galactosylation Promotes Hexamerization of Human IgG1, Leading to Enhanced Classical Complement Activation

(2021) Thijs L. J. van Osch et al.   JOURNAL OF IMMUNOLOGY

C1q binding to surface-bound IgG is stabilized by C1r2s2 proteases

(2021) Seline A. Zwarthoff et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The complement C5 inhibitor crovalimab in paroxysmal nocturnal hemoglobinuria

(2020) Alexander Röth et al.   BLOOD

Discovery of 4-((2S,4S)-4-Ethoxy-1-((5-methoxy-7-methyl-1H-indol-4-yl)methyl)piperidin-2-yl)benzoic Acid (LNP023), a Factor B Inhibitor Specifically Designed To Be Applicable to Treating a Diverse Array of Complement Mediated Diseases

(2020) Nello Mainolfi et al.   JOURNAL OF MEDICINAL CHEMISTRY

Clinical Effects of the Self-administered Subcutaneous Complement Inhibitor Zilucoplan in Patients With Moderate to Severe Generalized Myasthenia Gravis

(2020) James F. Howard et al.   JAMA Neurology

Interaction of the Factor H Family Proteins FHR-1 and FHR-5 With DNA and Dead Cells: Implications for the Regulation of Complement Activation and Opsonization

(2020) Éva Kárpáti et al.   Frontiers in Immunology

Safety, Tolerability and Efficacy of Narsoplimab, a Novel MASP-2 Inhibitor for the Treatment of IgA Nephropathy

(2020) Richard Lafayette et al.   Kidney International Reports

C5 Inhibitor Avacincaptad Pegol for Geographic Atrophy Due to Age-Related Macular Degeneration

(2020) Glenn J. Jaffe et al.   OPHTHALMOLOGY

Systemic complement activation is associated with respiratory failure in COVID-19 hospitalized patients

(2020) Jan C. Holter et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Sweet turning bitter: Carbohydrate sensing of complement in host defence and disease

(2020) Rachel Hevey et al.   BRITISH JOURNAL OF PHARMACOLOGY

Self versus Nonself Discrimination by the Soluble Complement Regulators Factor H and FHL-1

(2019) Arthur Dopler et al.   JOURNAL OF IMMUNOLOGY

CD20 and CD37 antibodies synergize to activate complement by Fc-mediated clustering

(2019) Simone C. Oostindie et al.   HAEMATOLOGICA

Novel MASP-2 inhibitors developed via directed evolution of human TFPI1 are potent lectin pathway inhibitors

(2019) Dávid Szakács et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Eculizumab in Aquaporin-4–Positive Neuromyelitis Optica Spectrum Disorder

(2019) Sean J. Pittock et al.   NEW ENGLAND JOURNAL OF MEDICINE

Clinical promise of next-generation complement therapeutics

(2019) Dimitrios C. Mastellos et al.   NATURE REVIEWS DRUG DISCOVERY

Revisiting the role of the innate immune complement system in ALS

(2019) Sandra E. Parker et al.   NEUROBIOLOGY OF DISEASE

Insights into IgM-mediated complement activation based on in situ structures of IgM-C1-C4b

(2019) Thomas H. Sharp et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A cryptic non–GPI-anchored cytosolic isoform of CD59 controls insulin exocytosis in pancreatic β-cells by interaction with SNARE proteins

(2019) Ewelina Golec et al.   FASEB JOURNAL

Cutting Edge: Role of MASP-3 in the Physiological Activation of Factor D of the Alternative Complement Pathway

(2019) Manabu Hayashi et al.   JOURNAL OF IMMUNOLOGY

The complement system in schizophrenia: where are we now and what’s next?

(2019) Julia J. Woo et al.   MOLECULAR PSYCHIATRY

Adipsin preserves beta cells in diabetic mice and associates with protection from type 2 diabetes in humans

(2019) Nicolás Gómez-Banoy et al.   NATURE MEDICINE

Complement dysregulation in the central nervous system during development and disease

(2019) John D. Lee et al.   SEMINARS IN IMMUNOLOGY

Successful treatment of a PNH patient non‐responsive to eculizumab with the novel complement C5 inhibitor coversin (nomacopan)

(2019) Saskia Schols et al.   BRITISH JOURNAL OF HAEMATOLOGY

Carbamylation reduces the capacity of IgG for hexamerization and complement activation

(2019) R. Lubbers et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Safety and efficacy of eculizumab in Guillain-Barré syndrome: a multicentre, double-blind, randomised phase 2 trial

(2018) Sonoko Misawa et al.   LANCET NEUROLOGY

CAR T cell–induced cytokine release syndrome is mediated by macrophages and abated by IL-1 blockade

(2018) Theodoros Giavridis et al.   NATURE MEDICINE

Efficacy and Safety of Lampalizumab for Geographic Atrophy Due to Age-Related Macular Degeneration

(2018) Frank G. Holz et al.   JAMA Ophthalmology

High Complement Factor H-Related (FHR)-3 Levels Are Associated With the Atypical Hemolytic-Uremic Syndrome-Risk Allele CFHR3*B

(2018) Richard B. Pouw et al.   Frontiers in Immunology

Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family

(2018) Pilar Sánchez-Corral et al.   Frontiers in Immunology

Developments in anti-complement therapy; from disease to clinical trial

(2018) Claire L. Harris et al.   MOLECULAR IMMUNOLOGY

Functional Characterization of Alternative and Classical Pathway C3/C5 Convertase Activity and Inhibition Using Purified Models

(2018) Seline A. Zwarthoff et al.   Frontiers in Immunology

Ravulizumab (ALXN1210) vs eculizumab in C5-inhibitor-experienced adult patients with PNH: the 302 study

(2018) Austin G. Kulasekararaj et al.   BLOOD

Ravulizumab (ALXN1210) vs eculizumab in adult patients with PNH naive to complement inhibitors: the 301 study

(2018) Jong Wook Lee et al.   BLOOD

Complement factor and T‐cell interactions during alloimmune inflammation in transplantation

(2018) Mohammad Afzal Khan et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Endogenous Natural Complement Inhibitor Regulates Cardiac Development

(2017) Simon A. Mortensen et al.   JOURNAL OF IMMUNOLOGY

Analysis of Factor D Isoforms in Malpuech–Michels–Mingarelli–Carnevale Patients Highlights the Role of MASP-3 as a Maturase in the Alternative Pathway of Complement

(2017) Rasmus Pihl et al.   JOURNAL OF IMMUNOLOGY

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study

(2017) James F Howard et al.   LANCET NEUROLOGY

Intracellular complement − the complosome − in immune cell regulation

(2017) Giuseppina Arbore et al.   MOLECULAR IMMUNOLOGY

Complement in ANCA-associated vasculitis: mechanisms and implications for management

(2017) Min Chen et al.   Nature Reviews Nephrology

CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis

(2017) Ahmet Ozen et al.   NEW ENGLAND JOURNAL OF MEDICINE

Decoding the Human Immunoglobulin G-Glycan Repertoire Reveals a Spectrum of Fc-Receptor- and Complement-Mediated-Effector Activities

(2017) Gillian Dekkers et al.   Frontiers in Immunology

Paroxysmal nocturnal haemoglobinuria

(2017) Anita Hill et al.   Nature Reviews Disease Primers

Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment

(2016) Sacha Zeerleder et al.   ANNALS OF MEDICINE

Factor H uptake regulates intracellular C3 activation during apoptosis and decreases the inflammatory potential of nucleosomes

(2016) M Martin et al.   CELL DEATH AND DIFFERENTIATION

Complement-coagulation crosstalk on cellular and artificial surfaces

(2016) Rebecca Wiegner et al.   IMMUNOBIOLOGY

A journey through the lectin pathway of complement-MBL and beyond

(2016) Peter Garred et al.   IMMUNOLOGICAL REVIEWS

Complement in disease: a defence system turning offensive

(2016) Daniel Ricklin et al.   Nature Reviews Nephrology

Complement therapeutic strategies in trauma, hemorrhagic shock and systemic inflammation – closing Pandora’s box?

(2016) Markus Huber-Lang et al.   SEMINARS IN IMMUNOLOGY

Autocrine Complement Inhibits IL10-Dependent T-cell-Mediated Antitumor Immunity to Promote Tumor Progression

(2016) Y. Wang et al.   Cancer Discovery

Hijacking Complement Regulatory Proteins for Bacterial Immune Evasion

(2016) Elise S. Hovingh et al.   Frontiers in Microbiology

MASP-3 is the exclusive pro-factor D activator in resting blood: the lectin and the alternative complement pathways are fundamentally linked

(2016) József Dobó et al.   Scientific Reports

Compstatin: a C3-targeted complement inhibitor reaching its prime for bedside intervention

(2015) Dimitrios C. Mastellos et al.   EUROPEAN JOURNAL OF CLINICAL INVESTIGATION

Complement Is a Central Mediator of Radiotherapy-Induced Tumor-Specific Immunity and Clinical Response

(2015) Laura Surace et al.   IMMUNITY

Complement Evasion Mediated by Enhancement of Captured Factor H: Implications for Protection of Self-Surfaces from Complement

(2015) Andrew P. Herbert et al.   JOURNAL OF IMMUNOLOGY

Complement-Coagulation Cross-Talk: A Potential Mediator of the Physiological Activation of Complement by Low pH

(2015) Hany Ibrahim Kenawy et al.   Frontiers in Immunology

Genetic and Pharmacologic Inhibition of Complement Impairs Endothelial Cell Function and Ablates Ovarian Cancer Neovascularization

(2015) Selene Nunez-Cruz et al.   NEOPLASIA

Complement C5a Receptor Facilitates Cancer Metastasis by Altering T-Cell Responses in the Metastatic Niche

(2014) S. K. Vadrevu et al.   CANCER RESEARCH

Identification of Factor H–like Protein 1 as the Predominant Complement Regulator in Bruch’s Membrane: Implications for Age-Related Macular Degeneration

(2014) Simon J. Clark et al.   JOURNAL OF IMMUNOLOGY

Intracellular sensing of complement C3 activates cell autonomous immunity

(2014) J. C. H. Tam et al.   SCIENCE

Complement Is Activated by IgG Hexamers Assembled at the Cell Surface

(2014) C. A. Diebolder et al.   SCIENCE

Crosstalk between the coagulation and complement systems in sepsis

(2014) Florea Lupu et al.   THROMBOSIS RESEARCH

Enhancement of Human Cancer Cell Motility and Invasiveness by Anaphylatoxin C5a via Aberrantly Expressed C5a Receptor (CD88)

(2013) H. Nitta et al.   CLINICAL CANCER RESEARCH

The novel complement inhibitor human CUB and Sushi multiple domains 1 (CSMD1) protein promotes factor I-mediated degradation of C4b and C3b and inhibits the membrane attack complex assembly

(2013) Astrid Escudero-Esparza et al.   FASEB JOURNAL

Intracellular Complement Activation Sustains T Cell Homeostasis and Mediates Effector Differentiation

(2013) M. Kathryn Liszewski et al.   IMMUNITY

Anaphylatoxin C5a Creates a Favorable Microenvironment for Lung Cancer Progression

(2012) Leticia Corrales et al.   JOURNAL OF IMMUNOLOGY

The complotype: dictating risk for inflammation and infection

(2012) Claire L. Harris et al.   TRENDS IN IMMUNOLOGY

Protection of Nonself Surfaces from Complement Attack by Factor H-Binding Peptides: Implications for Therapeutic Medicine

(2011) You-Qiang Wu et al.   JOURNAL OF IMMUNOLOGY

Complement in organ transplantation

(2010) Elham Asgari et al.   Current Opinion in Organ Transplantation

Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis

(2010) Daniel P Gale et al.   LANCET

Genome-wide association study identifies variants in the CFH region associated with host susceptibility to meningococcal disease

(2010) Sonia Davila et al.   NATURE GENETICS

Complement: coming full circle

(2009) Gaëlle Le Friec et al.   ARCHIVUM IMMUNOLOGIAE ET THERAPIAE EXPERIMENTALIS

Plaque complement activation and cognitive loss in Alzheimer's disease

(2008) David A Loeffler et al.   Journal of Neuroinflammation

Complement evasion by human pathogens

(2008) John D. Lambris et al.   NATURE REVIEWS MICROBIOLOGY