Results 21 to 30 of about 1,061 (120)

Low Levels of Factor H Family Proteins During Meningococcal Disease Indicate Systemic Processes Rather Than Specific Depletion by Neisseria meningitidis

Frontiers in Immunology, 2022
Neisseria meningitidis, the causative agent of meningococcal disease (MD), evades complement-mediated clearance upon infection by ‘hijacking’ the human complement regulator factor H (FH).
Anna E. van Beek, Anna E. van Beek, Richard B. Pouw, Richard B. Pouw, Victoria J. Wright, Neneh Sallah, David Inwald, Clive Hoggart, Mieke C. Brouwer, Rachel Galassini, John Thomas, Leo Calvo-Bado, Colin G. Fink, Ilse Jongerius, Ilse Jongerius, Martin Hibberd, Diana Wouters, Michael Levin, Taco W. Kuijpers, Taco W. Kuijpers   +19 more
doaj   +1 more source

Complement Factor H-Related Proteins FHR1 and FHR5 Interact With Extracellular Matrix Ligands, Reduce Factor H Regulatory Activity and Enhance Complement Activation

Frontiers in Immunology, 2022
Components of the extracellular matrix (ECM), when exposed to body fluids may promote local complement activation and inflammation. Pathologic complement activation at the glomerular basement membrane and at the Bruch’s membrane is implicated in renal ...
Alexandra Papp, Krisztián Papp, Barbara Uzonyi, Barbara Uzonyi, Marcell Cserhalmi, Ádám I. Csincsi, Zsóka Szabó, Zsófia Bánlaki, David Ermert, Zoltán Prohászka, Zoltán Prohászka, Anna Erdei, Anna Erdei, Viviana P. Ferreira, Anna M. Blom, Mihály Józsi, Mihály Józsi   +16 more
doaj   +1 more source

Contribución de variantes funcionales y cuantitativas del Factor H y las proteínas FHRs (Factor H-Related proteins) del Complemento en patología renal

Nefrología, 2022
Resumen: El sistema del Complemento protege al organismo frente a procesos infecciosos, tumorales y autoinmunes, y requiere una regulación muy estricta para evitar una activación excesiva o inespecífica. Entre los componentes reguladores del Complemento destaca el factor H (FH), que controla su activación en plasma y sobre la superficie de las células ...
Irene Gómez Delgado, Pilar Sánchez-Corral   +1 more
openaire   +2 more sources

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

Frontiers in Immunology, 2018
The alternative pathway is a continuously active surveillance arm of the complement system, and it can also enhance complement activation initiated by the classical and the lectin pathways.
Pilar Sánchez-Corral, Richard B. Pouw, Margarita López-Trascasa, Margarita López-Trascasa, Mihály Józsi, Mihály Józsi   +5 more
doaj   +1 more source

The OSE complotype and its clinical potential

Frontiers in Immunology, 2022
Cellular death, aging, and tissue damage trigger inflammation that leads to enzymatic and non-enzymatic lipid peroxidation of polyunsaturated fatty acids present on cellular membranes and lipoproteins.
Lejla Alic, Christoph J. Binder, Nikolina Papac-Milicevic   +2 more
doaj   +1 more source

Autoantibodies Against the Complement Regulator Factor H in the Serum of Patients With Neuromyelitis Optica Spectrum Disorder

Frontiers in Immunology, 2021
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system (CNS), characterized by pathogenic, complement-activating autoantibodies against the main water channel in the CNS, aquaporin 4 (AQP4 ...
Barbara Uzonyi, Barbara Uzonyi, Zsóka Szabó, Eszter Trojnár, Eszter Trojnár, Satu Hyvärinen, Katalin Uray, Helle H. Nielsen, Anna Erdei, Anna Erdei, T. Sakari Jokiranta, Zoltán Prohászka, Zoltán Prohászka, Zsolt Illes, Zsolt Illes, Mihály Józsi, Mihály Józsi, Mihály Józsi   +17 more
doaj   +1 more source

Selective Binding of Heparin/Heparan Sulfate Oligosaccharides to Factor H and Factor H-Related Proteins: Therapeutic Potential for C3 Glomerulopathies

Frontiers in Immunology, 2021
Complement dysregulation is characteristic of the renal diseases atypical hemolytic uremic syndrome (aHUS) and complement component 3 glomerulopathy (C3G).
Markus A. Loeven, Marissa L. Maciej-Hulme, Cansu Yanginlar, Melanie C. Hubers, Edwin Kellenbach, Mark de Graaf, Toin H. van Kuppevelt, Jack Wetzels, Ton J. Rabelink, Richard J. H. Smith, Johan van der Vlag   +10 more
doaj   +1 more source

Factor H-Related Protein 4 (FHR-4) drives complement dysregulation in AMD

, 2020
Background: Age-related macular degeneration (AMD) is a leading cause of blindness. Genetic studies reported strong associations at the CFH locus, with 8 independent signals across KCNT2, CFH, and CFHR1-5. How these variants impact protein expression and function remains to be disentangled. Recently,[for full text, please go to the a.m. URL]
Lorés de Motta, LL, Cipriani, V, He, F, Fathalla, D, McHarg, S, Bayatti, N, Acar, IE, Hoyng, CCB, Fauser, S, Moore, A, Yates, JRW, Morgan, P, de Jong, EK, den Hollander, AI, Bishop, PN, Clark, SJ   +15 more
openaire   +2 more sources

CFH and CFHR Copy Number Variations in C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis

Frontiers in Genetics, 2021
C3 Glomerulopathy (C3G) and Immune Complex-Mediated Membranoproliferative glomerulonephritis (IC-MPGN) are rare diseases characterized by glomerular deposition of C3 caused by dysregulation of the alternative pathway (AP) of complement.
Rossella Piras, Matteo Breno, Elisabetta Valoti, Marta Alberti, Paraskevas Iatropoulos, Caterina Mele, Elena Bresin, Roberta Donadelli, Paola Cuccarolo, Richard J. H. Smith, Ariela Benigni, Giuseppe Remuzzi, Marina Noris   +12 more
doaj   +1 more source

Complement Factor H-Related Protein 3 Serum Levels Are Low Compared to Factor H and Mainly Determined by Gene Copy Number Variation in CFHR3.

PLoS ONE, 2016
The major human complement regulator in blood, complement factor H (FH), has several closely related proteins, called FH-related (FHR) proteins. As all FHRs lack relevant complement regulatory activity, their physiological role is not well understood ...
Richard B Pouw, Mieke C Brouwer, Judy Geissler, Laurens V van Herpen, Sacha S Zeerleder, Walter A Wuillemin, Diana Wouters, Taco W Kuijpers   +7 more
doaj   +1 more source