Results 1 to 10 of about 610,146 (137)

Functional analyses of complement convertases using C3 and C5-depleted sera. [PDF]

PLoS ONE, 2012
C3 and C5 convertases are central stages of the complement cascade since they converge the different initiation pathways, augment complement activation by an amplification loop and lead to a common terminal pathway resulting in the formation of the ...
Marcin Okroj, Emelie Holmquist, Ben C King, Anna M Blom   +3 more
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C3-Glomerulopathy Autoantibodies Mediate Distinct Effects on Complement C3- and C5-Convertases [PDF]

Frontiers in Immunology, 2019
C3 glomerulopathy (C3G) is a severe kidney disease, which is caused by defective regulation of the alternative complement pathway. Disease pathogenesis is heterogeneous and is caused by both autoimmune and genetic factors.
Fei Zhao, Sara Afonso, Susanne Lindner, Andrea Hartmann, Ina Löschmann, Bo Nilsson, Kristina N. Ekdahl, Lutz T. Weber, Sandra Habbig, Gesa Schalk, Michael Kirschfink, Peter F. Zipfel, Peter F. Zipfel, Christine Skerka   +13 more
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A Cell-Based Assay to Measure the Activity of the Complement Convertases

Kidney International Reports
Introduction: The complement system serves as a crucial defense mechanism against invading pathogens; however, dysregulation of this system can result in harmful consequences.
Małgorzata Stasiłojć, Grzegorz Stasiłojć, Alicja Kuźniewska, Santiago Rodriguez de Córdoba, Marcin Okrój   +4 more
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Moss-derived recombinant Factor H, CPV-104, effectively antagonizes alternative pathway C3/C5 convertases stabilization by NeFs from patients with primary C3 glomerulopathy [PDF]

Frontiers in Immunology
BackgroundC3 glomerulopathy (C3G) is a rare kidney disease caused by uncontrolled activation of the complement alternative pathway (AP), frequently driven by nephritic factors (NeFs) that stabilize the AP C3 and C5 convertases. NeFs are detected in up to
Zahra Imanifard, Francesca Penati, Sofia Padoa, Paulina Dabrowska-Schlepp, Elena Bresin, Tobia Peracchi, Ariela Benigni, Giuseppe Remuzzi, Marina Noris, Roberta Donadelli   +9 more
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Proprotein Convertases and the Complement System

Frontiers in Immunology, 2022
Proteins destined for secretion - after removal of the signal sequence - often undergo further proteolytic processing by proprotein convertases (PCs).
József Dobó, Andrea Kocsis, Ráhel Dani, Péter Gál   +3 more
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Properdin deficiency or anti-properdin treatment ameliorates disease in the C3 gain-of-function mouse model of atypical haemolytic uraemic syndrome [PDF]

Frontiers in Immunology
IntroductionWe have previously shown that the C3D1115N mouse, engineered around a single point mutation in C3 associated with atypical haemolytic uraemic syndrome (aHUS) in man, fully recapitulates the clinical disease. In this study, we investigated the
Grace Mallett, Grace Mallett, Ola Kamala, Ola Kamala, Isabel Y. Pappworth, Isabel Y. Pappworth, Kate Smith-Jackson, Kate Smith-Jackson, Beth G. Gibson, Beth G. Gibson, Muhammad Asif, Muhammad Asif, Harriet Denton, Harriet Denton, Charlotte Cox, Patrick Walsh, Patrick Walsh, David Kavanagh, David Kavanagh, Kevin J. Marchbank, Kevin J. Marchbank   +20 more
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Stringent regulation of complement lectin pathway C3/C5 convertase by C4b-binding protein (C4BP) [PDF]

Molecular Immunology, 2009
The complement lectin pathway, an essential component of the innate immune system, is geared for rapid recognition of infections as each C4b deposited via this pathway is capable of forming a C3/C5 convertase. In the present study, role of C4b-binding protein (C4BP) in regulating the lectin pathway C3/C5 convertase assembled on zymosan and sheep ...
Nenoo, Rawal, Rema, Rajagopalan, Veena P, Salvi   +2 more
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Alternative pathway of complement: recruitment of precursor properdin by the labile C3/C5 convertase and the potentiation of the pathway. [PDF]

The Journal of experimental medicine, 1976
In this study the physiological role of properdin and the differential subunit composition of the solid phase enzymes of the pathway have been explored. Cell-bound C3 and C5 convertase differ in their C3b requirement. Apparently one molecule of C3b is sufficient to allow formation of C3 convertase (C3b,B), whereas two or more are required for ...
R G, Medicus, O, Götze, H J, Müller-Eberhard   +2 more
openaire   +2 more sources

Cryo-EM structures of Trypanosoma brucei gambiense ISG65 with human complement C3 and C3b and their roles in alternative pathway restriction

Nature Communications, 2023
African Trypanosomes have developed elaborate mechanisms to escape the adaptive immune response, but little is known about complement evasion particularly at the early stage of infection.
Hagen Sülzen, Jakub Began, Arun Dhillon, Sami Kereïche, Petr Pompach, Jitka Votrubova, Farnaz Zahedifard, Adriana Šubrtova, Marie Šafner, Martin Hubalek, Maaike Thompson, Martin Zoltner, Sebastian Zoll   +12 more
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The role of the complement system in kidney glomerular capillary thrombosis

Frontiers in Immunology, 2022
The complement system is part of the innate immune system. The crucial step in activating the complement system is the generation and regulation of C3 convertase complexes, which are needed to generate opsonins that promote phagocytosis, to generate C3a ...
Yoko Yoshida, Hiroshi Nishi
doaj   +1 more source