Results 61 to 70 of about 8,202 (220)

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

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

Characterization of the human properdin gene [PDF]

Biochemical Journal, 1992
A cosmid clone containing the complete coding sequence of the human properdin gene has been characterized. The gene is located at one end of the approximately 40 kb cosmid insert and approximately 8.2 kb of the sequence data have been obtained from this region.
Nolan, K. F., Kaluz, S., Higgins, J. M G, Goundis, D., Reid, K. B M   +4 more
openaire   +5 more sources

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

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‐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

A molecular concept of the properdin pathway. [PDF]

Proceedings of the National Academy of Sciences, 1976
The sequential events of the properdin system were analyzed. Properdin-depleted serum allows the formation of a Factor B- and D-dependent C3 convertase. This enzyme, called the properdin-receptor-forming enzyme, was shown to utilize a novel serum component, the initiating factor.
Rudolf G. Medicus, Otto Gotze, Hans J. Müller-Eberhard, Robert D. Schreiber   +3 more
openaire   +3 more sources

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

Case report: A novel high‐dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy

The Journal of Dermatology, Volume 51, Issue 12, Page 1665-1668, December 2024.
Abstract Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid‐sparing immunosuppressants, or intravenous monoclonal anti–CD20‐antibody therapy.
Nadine Wiedenmayer, Katharina Hogrefe, Silvia Mihalceanu, Julia K. Winkler, Alexander H. Enk   +4 more
wiley   +1 more source