Results 21 to 30 of about 28,235,749 (276)

Beyond factor H: The impact of genetic-risk variants for age-related macular degeneration on circulating factor-H-like 1 and factor-H-related protein concentrations. [PDF]

Am J Hum Genet, 2021
Age-related macular degeneration (AMD) is a leading cause of vision loss; there is strong genetic susceptibility at the complement factor H (CFH) locus. This locus encodes a series of complement regulators: factor H (FH), a splice variant factor-H-like 1
Cipriani V, Tierney A, Griffiths JR, Zuber V, Sergouniotis PI, Yates JRW, Moore AT, Bishop PN, Clark SJ, Unwin RD.   +9 more
europepmc   +2 more sources

Factor H-Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy. [PDF]

J Am Soc Nephrol, 2022
Significance Statement Mutations in factor H–related protein 1 (FHR-1) that result in duplication of its dimerization domain associate with the chronic renal disease C3 glomerulopathy (C3G), which is characterized by complement dysregulation. The molecular basis for this association is only partially understood.
Márquez-Tirado B, Gutiérrez-Tenorio J, Tortajada A, Lucientes Continente L, Caravaca-Fontán F, Malik TH, Roldán Montero R, Elías S, Saiz Gonzalez A, Saiz Gonzalez A, Fernández-Juarez G, Sánchez-Corral P, Pickering MC, Praga M, Rodríguez de Córdoba S, Goicoechea de Jorge E.   +15 more
europepmc   +5 more sources

The human factor H protein family – an update

Frontiers in Immunology
Complement is an ancient and complex network of the immune system and, as such, it plays vital physiological roles, but it is also involved in numerous pathological processes.
Noémi Sándor, Noémi Sándor, Andrea E. Schneider, Alexandra T. Matola, Veronika H. Barbai, Dániel Bencze, Hani Hashim Hammad, Alexandra Papp, Dorottya Kövesdi, Dorottya Kövesdi, Barbara Uzonyi, Barbara Uzonyi, Mihály Józsi, Mihály Józsi   +13 more
doaj   +2 more sources

The Impact of Complement Factor H-Related Protein Gene Deletions on Kidney Transplantation. [PDF]

Scand J Immunol
ABSTRACT We recently reported that a homozygous deletion in the complement factor H-related ( CFHR) locus predisposed kidney transplant patients to rejection. As donors carried intact genes, the susceptibility may have resulted from alloimmune reaction to FHR proteins.
Markkinen S, Lokki AI, Helanterä I, Ritari J, Partanen J, Meri S, Hyvärinen K.   +6 more
europepmc   +4 more sources

A Family Affair: Addressing the Challenges of Factor H and the Related Proteins

Frontiers in Immunology, 2021
Inflammation is a common denominator of diseases. The complement system, an intrinsic part of the innate immune system, is a key driver of inflammation in numerous disorders.
Felix Poppelaars, Elena Goicoechea de Jorge, Ilse Jongerius, Ilse Jongerius, Antje J. Baeumner, Mark-Steven Steiner, Mihály Józsi, Mihály Józsi, Erik J. M. Toonen, Diana Pauly, Diana Pauly, the SciFiMed consortium   +11 more
doaj   +1 more source

The Factor H protein family: The switchers of the complement alternative pathway [PDF]

, 2022
CRUE-CSIC (Acuerdos Transformativos 2022)The factor H (FH) protein family is emerging as a complex network of proteins controlling the fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range of diseases including ...
Márquez Tirado, Bárbara, Lucientes Continente, Laura, Márquez-Tirado, Bárbara, Goicoechea de Jorge, Elena, Bárbara Márquez‐Tirado, Lucientes-Continente, Laura, Laura Lucientes‐Continente, Elena Goicoechea de Jorge   +7 more
core   +1 more source

Modulation of the Alternative Pathway of Complement by Murine Factor H–Related Proteins [PDF]

The Journal of Immunology, 2018
Abstract Factor H (FH) is a key alternative pathway regulator that controls complement activation both in the fluid phase and on specific cell surfaces, thus allowing the innate immune response to discriminate between self and foreign pathogens.
Alexandra H Antonioli, Janice White, Frances Crawford, Brandon Renner, Kevin J Marchbank, Jonathan P Hannan, Joshua M Thurman, Philippa Marrack, V Michael Holers   +8 more
openaire   +5 more sources

Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children [PDF]

Frontiers in Immunology, 2018
Complement is activated as part of the innate immune defense against invading pathogens. Also, it helps to remove apoptotic debris and immune complexes from the circulation. Impaired complement function due to aberrant plasma levels of complement proteins may be indicative for complement-mediated diseases or can be involved in susceptibility for ...
Anna E. van Beek, Anna E. van Beek, Angela Kamp, Simone Kruithof, Ed J. Nieuwenhuys, Diana Wouters, Ilse Jongerius, Theo Rispens, Taco W. Kuijpers, Taco W. Kuijpers, Kyra A. Gelderman   +10 more
openaire   +4 more sources

Case Report: Severe Complement-Mediated Thrombotic Microangiopathy in IgG4-Related Disease Secondary to Anti-Factor H IgG4 Autoantibodies

Frontiers in Immunology, 2021
ObjectiveTo first describe and estimate the potential pathogenic role of Ig4 autoantibodies in complement-mediated thrombotic microangiopathy (TMA) in a patient with IgG4-related disease (IgG4-RD).MethodsThis study is a case report presenting a ...
Gautier Breville, Gautier Breville, Ido Zamberg, Ido Zamberg, Salima Sadallah, Caroline Stephan, Belen Ponte, Jörg D. Seebach   +7 more
doaj   +1 more source

Complement Factor H Family Proteins Modulate Monocyte and Neutrophil Granulocyte Functions

Frontiers in Immunology, 2021
Besides being a key effector arm of innate immunity, a plethora of non-canonical functions of complement has recently been emerging. Factor H (FH), the main regulator of the alternative pathway of complement activation, has been reported to bind to ...
Éva Kárpáti, Mariann Kremlitzka, Noémi Sándor, Noémi Sándor, Dávid Hajnal, Andrea E. Schneider, Mihály Józsi, Mihály Józsi   +7 more
doaj   +1 more source