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Anti-complement-factor H-associated glomerulopathies

Nature Reviews Nephrology, 2016
Atypical haemolytic uraemic syndrome (aHUS), an important cause of acute kidney injury, is characterized by dysregulation of the complement pathway, frequent need for dialysis, and progression to end-stage renal disease. Autoantibodies against complement factor H (FH), the main plasma regulatory protein of the alternative pathway of the complement ...
Marie-Agnes Dragon, Durey   +3 more
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Complement factor H: a guardian within?

Kidney International, 2021
The glomerular endothelium produces the key complement regulator factor H (FH), but its role in the endothelial cells protection and functional integrity is unclear. In this edition of Kidney International, Mahajan et al. demonstrate that the endothelial-intrinsic FH is important for the cytoskeletal architecture, monolayer integrity, proliferation ...
Idris Boudhabhay, Lubka T. Roumenina
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Complement regulator factor H in multiple sclerosis

Journal of Cellular Biochemistry, 2011
A recent proteomic study published in this journal demonstrated lower cerebrospinal fluid (CSF) expression of factor H (fH), an important complement regulator, along with two other complement proteins, in active multiple sclerosis (MS) patients. We have previously demonstrated raised serum fH levels in MS and here, an extended analysis, quantifying fH ...
Gillian, Ingram   +4 more
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Ancient Origin of Human Complement Factor H

Journal of Molecular Evolution, 1998
We studied the evolutionary history of two homologous proteins of the human complement system, factor H (FH) and the alpha chain of the C4b binding protein (C4bpalpha), and included in this study the related proteins from the barred sand bass (P. nebulifer) and the nematode C. elegans. Phylogenetic trees inferred from individual short consensus repeats
J, Krushkal, C, Kemper, I, Gigli
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Sequence polymorphism of human complement factor H

Immunogenetics, 1988
Factor H is a major regulatory protein of the complement system. The complete cDNA coding sequence has been derived from overlapping clones, and a polymorphism at base 1277 has been characterized. In four clones there is a T at nucleotide 1277 and in two others there is a C.
Day, Anthony J.   +3 more
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Complement factor H and hemolytic uremic syndrome

International Immunopharmacology, 2001
Factor H is a 150 kDa single chain plasma glycoprotein that plays a pivotal role in the regulation of the alternative pathway of complement. Primary sequence analysis reveals a structural organization of this plasma protein, in 20 homologous units, called Short Consensus Repeats (SCRs), each about 60 amino acids long.
Zipfel, Peter F.   +6 more
openaire   +3 more sources

Complement Factor H: Physiology and Pathophysiology

Seminars in Thrombosis and Hemostasis, 2001
The human plasma protein factor H, which is a multifunctional, multidomain protein, acts as a central regulator of the complement system. In addition to its complement regulatory activities, factor H has multiple physiological activities and 1) acts as an extracellular matrix component, 2) binds to cellular receptors of the integrin type, and 3 ...
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Plasma Complement factor H in Alzheimer's Disease

Journal of Alzheimer's Disease, 2015
Biomarkers for Alzheimer's disease (AD) should meet several criteria, including simplicity of testing. Inappropriate activation of the complement cascade has been implicated in the pathogenesis of AD. Complement factor H (CFH) is a regulator of the cascade, but studies on plasma CFH levels in AD have provided mixed results.
Williams, Michael A.   +5 more
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Urinary Complement Factor H in Renal Disease

Nephron, 2002
<i>Background:</i> Complement factor H (hCFH) plays a key inhibitory role in the control of the alternative complement pathway. We examined whether urinary hCFH (U-hCFH) levels is useful as an indirect indicator of renal damage. <i>Methods:</i> Urine samples were obtained from 104 patients with renal disease. Urine was collected
Mariko, Tamano   +5 more
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Elevated Complement Factor H Levels in Asthmatic Sputa

Journal of Clinical Immunology, 2012
The alternative pathway of the complement system is known to play a role in the generation of asthmatic airway inflammation, but its regulatory complement protein, factor H has not been investigated in this disease.Our aim was to determine the local bronchial complement factor H (CFH) levels in asthma, and to investigate its relationship with ...
Weiszhár, Z.   +7 more
openaire   +4 more sources

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