Results 71 to 80 of about 10,182 (218)

Two New Kindreds with Complete Factor D Deficiency

open access: yesEuropean 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   +10 more
wiley   +1 more source

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

open access: yesProtein 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   +5 more
wiley   +1 more source

Study of the variables associated with local complement activation in IgA nephropathy

open access: yesNefrología (English Edition), 2017
Objectives: 1. To identify the variables that are associated with urinary levels of properdin, MBL, C4d, and C5b-9 in patients with idiopathic IgA nephropathy. 2. To analyse whether urinary levels of MBL and/or C4d are useful for identifying the presence
Alfons Segarra-Medrano   +6 more
doaj   +1 more source

Biotic and abiotic factors of bactericidal properties variability of ruminant saliva

open access: yesBIO Web of Conferences, 2020
The microflora of the oral cavity of domestic ruminants, presented by transitive gram-negative species of bacteria (cocci and sticks) in cows, transient and obligate gram-positive (usually cocci) bacteria in sheep was studied. The lowest contamination of
Gilmutdinov Rustam Y.   +3 more
doaj   +1 more source

CSF proteomics reveals changes in myelin and synaptic biology after Spectris treatment

open access: yesAlzheimer's &Dementia: Translational Research &Clinical Interventions, Volume 11, Issue 1, January-March 2025.
Abstract INTRODUCTION Brain steady‐state gamma oscillations evoked using a non‐invasive medical device (Spectris) have shown potential clinical benefits in patients with mild–moderate Alzheimer's disease (AD), including reduced functional and cognitive decline, reduced brain volume and myelin loss, and increased brain functional connectivity.
Mihály Hajós   +10 more
wiley   +1 more source

Blocking Properdin Prevents Complement-Mediated Hemolytic Uremic Syndrome and Systemic Thrombophilia.

open access: yesJournal of the American Society of Nephrology, 2018
Background Properdin (P) is a positive regulator of the alternative pathway of complement activation. Although P inhibition is expected and has been shown to ameliorate the alternative pathway of complement-mediated tissue injury in several disease ...
Yoshiyasu Ueda   +7 more
semanticscholar   +1 more source

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

open access: yesEuropean 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

The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy?

open access: yesPediatric nephrology (Berlin, West), 2018
Properdin is known as the only positive regulator of the complement system. Properdin promotes the activity of this defense system by stabilizing its key enzymatic complexes: the complement alternative pathway (AP) convertases. Besides, some studies have
Marloes A. H. M. Michels   +3 more
semanticscholar   +1 more source

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

open access: yesGlia, 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   +6 more
wiley   +1 more source

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

open access: yesThe 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   +4 more
wiley   +1 more source

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