Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children [PDF]
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.
Anna E. van Beek +10 more
doaj +6 more sources
Selective Binding of Heparin/Heparan Sulfate Oligosaccharides to Factor H and Factor H-Related Proteins: Therapeutic Potential for C3 Glomerulopathies [PDF]
Complement dysregulation is characteristic of the renal diseases atypical hemolytic uremic syndrome (aHUS) and complement component 3 glomerulopathy (C3G).
Markus A. Loeven +10 more
doaj +2 more sources
Complement Factor H-Related Proteins FHR1 and FHR5 Interact With Extracellular Matrix Ligands, Reduce Factor H Regulatory Activity and Enhance Complement Activation [PDF]
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 +16 more
doaj +2 more sources
Identification of novel blood-borne soluble binding partners of factor H-related proteins [PDF]
The deposition of circulating complement factor H-related (FHR) proteins in tissues around the body has been implicated in a series of complement-mediated diseases.
Jiaqi Tang +8 more
doaj +2 more sources
Factor H-related proteins determine complement-activating surfaces [PDF]
Complement factor H-related proteins (FHRs) are strongly associated with different diseases involving complement dysregulation, which suggests a major role for these proteins regulating complement activation. Because FHRs are evolutionarily and structurally related to complement inhibitor factor H (FH), the initial assumption was that the FHRs are also
Józsi, Mihály +4 more
openaire +6 more sources
Development and Characterization of Novel ELISAs for the Specific Quantification of the Factor H-Related Proteins 2, 3, 4, and 5 [PDF]
Introduction: The complement system’s alternative pathway relies on factor H (FH) for immune homeostasis. Next to FH, a group of highly similar proteins was described known as the FH-related (FHR) proteins.
Mara van Rossum +14 more
doaj +2 more sources
Human factor H-related protein 2 (CFHR2) regulates complement activation. [PDF]
Mutations and deletions within the human CFHR gene cluster on chromosome 1 are associated with diseases, such as dense deposit disease, CFHR nephropathy or age-related macular degeneration. Resulting mutant CFHR proteins can affect complement regulation.
Hannes U Eberhardt +10 more
doaj +4 more sources
Factor H-related protein 1 in systemic lupus erythematosus [PDF]
BackgroundFactor H (FH) is a major soluble inhibitor of the complement system and part of a family comprising five related proteins (FHRs 1–5). Deficiency of FHR1 was described to be linked to an elevated risk of systemic lupus erythematosus (SLE).
Jessica S. Kleer +9 more
doaj +3 more sources
Factor H related proteins modulate complement activation on kidney cells. [PDF]
Complement activation at a particular location is determined by the balance of activating and inhibitory proteins. Factor H is a key regulator of the alternative pathway of complement, and genetic or acquired impairments in Factor H are associated with glomerular injury. The human Factor H-related proteins (FHRs) comprise a family of five proteins that
Renner B +12 more
europepmc +4 more sources
Role of factor H-related protein 3 in Pseudomonas aeruginosa bloodstream infections
Pseudomonas aeruginosa is a leading cause of nosocomial bloodstream infections. The outcome of these infections depends on the virulence of the microorganism as well as host-related conditions and factors.
Alex González-Alsina +7 more
doaj +4 more sources

