Results 41 to 50 of about 5,264 (155)

Two New Kindreds with Complete Factor D Deficiency

European Journal of Immunology, Volume 55, Issue 3, March 2025.
The complement system plays a crucial role in immune defense, and factor D deficiency is a rare immunodeficiency associated with life‐threatening bacterial infections. We report two new cases of complete factor D deficiency, including the first described in a Down syndrome patient, highlighting the need to investigate complement deficiencies in ...
Mathilde Puel, Kenza Rwayane, Paula Vieira Martins, Marwa Chbihi, Frédéric Rieux‐Laucat, Jérémie Rosain, Eric Jeziorski, Bertrand Boisson, Jean‐Laurent Casanova, Véronique Frémeaux‐Bacchi, Carine El Sissy   +10 more
wiley   +1 more source

Protective role for properdin in progression of experimental murine atherosclerosis.

PLoS ONE, 2014
Genetic, dietary and immune factors contribute to the pathogenesis of atherosclerosis in humans and mice. Complement activation is an integral part of the innate immune defence but also shapes cellular responses and influences directly triglyceride ...
Tanja Steiner, Lorenza Francescut, Simon Byrne, Timothy Hughes, Archana Jayanthi, Irina Guschina, John Harwood, Katherine Cianflone, Cordula Stover, Sheila Francis   +9 more
doaj   +1 more source

Complement alternative pathway activation in human nonalcoholic steatohepatitis. [PDF]

PLoS ONE, 2014
The innate immune system plays a major role in the pathogenesis of nonalcoholic steatohepatitis (NASH). Recently we reported complement activation in human NASH.
Filip M Segers, Froukje J Verdam, Charlotte de Jonge, Bas Boonen, Ann Driessen, Ronit Shiri-Sverdlov, Nicole D Bouvy, Jan Willem M Greve, Wim A Buurman, Sander S Rensen   +9 more
doaj   +1 more source

Methods for detecting, building, and improving tryptophan mannosylation in glycoprotein structures

Protein Science, Volume 34, Issue 2, February 2025.
Abstract Tryptophan mannosylation, the covalent addition of an α‐ᴅ‐mannose sugar to a tryptophan side chain, is a post‐translational modification (PTM) that can affect protein stability, folding, and interactions. Compared to other forms of protein glycosylation, it is relatively uncommon but is affected by conformational anomalies and modeling errors ...
Lou Holland, Phuong Thao Pham, Haroldas Bagdonas, Jordan S. Dialpuri, Lucy C. Schofield, Jon Agirre   +5 more
wiley   +1 more source

Complement activation in Hidradenitis suppurativa: Covert low-grade inflammation or innocent bystander?

Frontiers in Immunology, 2022
Hidradenitis suppurativa (HS) is a chronic auto-inflammatory skin disease with a complex and multifactorial pathogenesis involving both the innate and adaptive immune system.
K. R. van Straalen, K. Dudink, P. Aarts, H. H. van der Zee, T. P. P. van den Bosch, J. Giang, E. P. Prens, E. P. Prens, J. Damman   +8 more
doaj   +1 more source

Alternative Complement Pathway in Carotid Atherosclerosis: Low Plasma Properdin Levels Associate With Long‐Term Cardiovascular Mortality

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Complement activation may promote atherosclerosis. Yet, data on the to which extent complement, and more specifically the alternative complement pathway, is activated in patients with carotid atherosclerosis and related to adverse outcome in ...
Mieke C. Louwe, Chrysostomi Gialeli, Annika E. Michelsen, Sverre Holm, Andreas Edsfeldt, Karolina Skagen, Tove Lekva, Maria Belland Olsen, Vigdis Bjerkeli, Therese Schjørlien, Kristine Stø, Xiang Yi Kong, Tuva B. Dahl, Per H. Nilsson, Peter Libby, Pål Aukrust, Tom Eirik Mollnes, Thor Ueland, Mona Skjelland, Isabel Gonçalves, Bente Halvorsen   +20 more
doaj   +1 more source

Complement‐targeted therapeutics: Are we there yet, or just getting started?

European Journal of Immunology, Volume 54, Issue 12, December 2024.
After a hesitant start, the number of available therapeutics that target the human complement system has surged from one to a dozen drugs in less than 20 years. Meanwhile, the clinical arsenal encompasses treatment modalities directed to several targets for the therapy of a steadily growing spectrum of complement‐related disorders. Abstract Therapeutic
Daniel Ricklin
wiley   +1 more source

Decreased sialylation elicits complement‐related microglia response and bipolar cell loss in the mouse retina

Glia, Volume 72, Issue 12, Page 2295-2312, December 2024.
Main Points Hyposialylation in Gne+/‐ mice induced loss of retinal rod bipolar cells. Hyposialylation triggered complement, inflammation and apoptosis transcriptome pathways in the retina. Crossing Gne+/‐ with C3‐deficient mice prevented the retinal changes.
German Cuevas‐Rios, Tawfik Abou Assale, Jannis Wissfeld, Annemarie Bungartz, Julia Hofmann, Thomas Langmann, Harald Neumann   +6 more
wiley   +1 more source

Human Properdin Opsonizes Nanoparticles and Triggers a Potent Pro-inflammatory Response by Macrophages without Involving Complement Activation

Frontiers in Immunology, 2018
Development of nanoparticles as tissue-specific drug delivery platforms can be considerably influenced by the complement system because of their inherent pro-inflammatory and tumorigenic consequences.
Lubna Kouser, Basudev Paudyal, Basudev Paudyal, Anuvinder Kaur, Gudrun Stenbeck, Lucy A. Jones, Suhair M. Abozaid, Cordula M. Stover, Emmanuel Flahaut, Robert B. Sim, Uday Kishore   +10 more
doaj   +1 more source

Unraveling the Molecular Mechanisms Underlying Complement Dysregulation by Nephritic Factors in C3G and IC-MPGN

Frontiers in Immunology, 2018
Membranoproliferative glomerulonephritis (MPGN) was recently classified as C3 glomerulopathies (C3G), and immune-complex (IC) mediated MPGN. Dysregulation of the complement alternative pathway, driven by acquired and/or genetic defects, plays a ...
Roberta Donadelli, Patrizia Pulieri, Rossella Piras, Paraskevas Iatropoulos, Elisabetta Valoti, Ariela Benigni, Giuseppe Remuzzi, Giuseppe Remuzzi, Giuseppe Remuzzi, Marina Noris   +9 more
doaj   +1 more source