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Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
by
Pouw, Richard B.
, Józsi, Mihály
, Sánchez-Corral, Pilar
, López-Trascasa, Margarita
in
Age
/ age-related macular degeneration
/ Alternative pathway
/ atypical hemolytic uremic syndrome
/ C3 glomerulopathy
/ Complement activation
/ complement de-regulation
/ Complement system
/ factor H
/ Genes
/ Genetic diversity
/ H protein
/ Hemolytic uremic syndrome
/ IgA nephropathy
/ Immune system
/ Immunoglobulin A
/ Immunoglobulins
/ Immunology
/ Kidney diseases
/ Lectins
/ Ligands
/ Macular degeneration
/ Physiology
/ Proteins
/ Regulation
/ Regulatory proteins
2018
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Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
by
Pouw, Richard B.
, Józsi, Mihály
, Sánchez-Corral, Pilar
, López-Trascasa, Margarita
in
Age
/ age-related macular degeneration
/ Alternative pathway
/ atypical hemolytic uremic syndrome
/ C3 glomerulopathy
/ Complement activation
/ complement de-regulation
/ Complement system
/ factor H
/ Genes
/ Genetic diversity
/ H protein
/ Hemolytic uremic syndrome
/ IgA nephropathy
/ Immune system
/ Immunoglobulin A
/ Immunoglobulins
/ Immunology
/ Kidney diseases
/ Lectins
/ Ligands
/ Macular degeneration
/ Physiology
/ Proteins
/ Regulation
/ Regulatory proteins
2018
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Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
by
Pouw, Richard B.
, Józsi, Mihály
, Sánchez-Corral, Pilar
, López-Trascasa, Margarita
in
Age
/ age-related macular degeneration
/ Alternative pathway
/ atypical hemolytic uremic syndrome
/ C3 glomerulopathy
/ Complement activation
/ complement de-regulation
/ Complement system
/ factor H
/ Genes
/ Genetic diversity
/ H protein
/ Hemolytic uremic syndrome
/ IgA nephropathy
/ Immune system
/ Immunoglobulin A
/ Immunoglobulins
/ Immunology
/ Kidney diseases
/ Lectins
/ Ligands
/ Macular degeneration
/ Physiology
/ Proteins
/ Regulation
/ Regulatory proteins
2018
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Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
Journal Article
Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
2018
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Overview
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. Various membrane-bound and plasma regulatory proteins control the activation of the potentially deleterious complement system. Among the regulators, the plasma glycoprotein factor H (FH) is the main inhibitor of the alternative pathway and its powerful amplification loop. FH belongs to a protein family that also includes FH-like protein 1 and five factor H-related (FHR-1 to FHR-5) proteins. Genetic variants and abnormal rearrangements involving the FH protein family have been linked to numerous systemic and organ-specific diseases, including age-related macular degeneration, and the renal pathologies atypical hemolytic uremic syndrome, C3 glomerulopathies, and IgA nephropathy. This review covers the known and recently emerged ligands and interactions of the human FH family proteins associated with disease and discuss the very recent experimental data that suggest FH-antagonistic and complement-activating functions for the FHR proteins.
Publisher
Frontiers Media SA,Frontiers Media S.A
Subject
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