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CFH and CFHR structural variants in atypical Hemolytic Uremic Syndrome: Prevalence, genomic characterization and impact on outcome

open access: yesFrontiers in Immunology, 2023
IntroductionAtypical hemolytic uremic syndrome (aHUS) is a rare disease that manifests with microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, and is associated with dysregulation of the alternative complement pathway.
Rossella Piras   +11 more
doaj   +1 more source

Hypoglycemia-induced changes in complement pathways in type 2 diabetes

open access: yesAtherosclerosis Plus, 2021
Background and aims: An association between hypoglycaemia and adverse cardiovascular events has been suggested from longitudinal and retrospective cohort studies. The complement pathway proteins in hypoglycemia are not well studied. Here, we hypothesized
Abu Saleh Md Moin   +6 more
doaj   +1 more source

Case report: Novel FHR2 variants in atypical Hemolytic Uremic Syndrome: A case study of a translational medicine approach in renal transplantation

open access: yesFrontiers in Immunology, 2022
Atypical hemolytic–uremic syndrome (aHUS) is a severe thrombotic microangiopathy in which kidney involvement is common. aHUS can be due to either genetic or acquired abnormalities, with most abnormalities affecting the alternative complement pathway ...
Emma Diletta Stea   +11 more
doaj   +1 more source

Complement Factor H-Related Protein 4A Is the Dominant Circulating Splice Variant of CFHR4

open access: yesFrontiers in Immunology, 2018
Recent research has elucidated circulating levels of almost all factor H-related (FHR) proteins. Some of these proteins are hypothesized to act as antagonists of the important complement regulator factor H (FH), fine-tuning complement regulation on human
Richard B. Pouw   +8 more
doaj   +1 more source

Complement factor H-related proteins CFHR2 and CFHR5 represent novel ligands for the infection-associated CRASP proteins of Borrelia burgdorferi. [PDF]

open access: yesPLoS ONE, 2010
One virulence property of Borrelia burgdorferi is its resistance to innate immunity, in particular to complement-mediated killing. Serum-resistant B. burgdorferi express up to five distinct complement regulator-acquiring surface proteins (CRASP) which ...
Corinna Siegel   +10 more
doaj   +1 more source

Human complement factor H. Two factor H proteins are derived from alternatively spliced transcripts [PDF]

open access: yes, 1991
The human complement factor H is an important component in the control of the alternative pathway of complement activation. We have previously shown that at least three factor H homologous mRNA species of 4.3 kb, 1.8 kb and 1.4 kb in length are ...
Alsenz   +23 more
core   +1 more source

The complement factor H‐related proteins

open access: yesImmunological Reviews, 2016
SummaryThe role of the complement factor H‐related (FHR) proteins in homeostasis, pathogen defense, and autoimmune disease has recently attracted considerable interest. We highlight the exciting research that has contributed to our understanding of the FHR protein family. Unlike factor H, a potent negative regulator of complement C3 activation, the FHR
Medjeral-Thomas, N, Pickering, MC
openaire   +4 more sources

Contribution of the infection-associated complement regulator-acquiring surface protein 4 (ErpC) to complement resistance of Borrelia burgdorferi [PDF]

open access: yes, 2012
Borrelia burgdorferi evades complement-mediated killing by interacting with complement regulators through distinct complement regulator-acquiring surface proteins (CRASPs).
Hammerschmidt, Claudia   +6 more
core   +3 more sources

The Murine Factor H-Related Protein FHR-B Promotes Complement Activation [PDF]

open access: yesFrontiers in Immunology, 2017
Factor H-related (FHR) proteins consist of varying number of complement control protein domains that display various degrees of sequence identity to respective domains of the alternative pathway complement inhibitor factor H (FH). While such FHR proteins are described in several species, only human FHRs were functionally investigated.
Cserhalmi, M   +6 more
openaire   +5 more sources

Factor H-Related (FHR)-1 and FHR-2 Form Homo- and Heterodimers, while FHR-5 Circulates Only As Homodimer in Human Plasma

open access: yesFrontiers in Immunology, 2017
The complement factor H-related (FHR) proteins are hypothesized to fine-tune the regulatory role of complement factor H (FH) in the alternative pathway of the complement system.
Anna E. van Beek   +13 more
doaj   +1 more source

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