C3 Glomerulopathy (C3G) and Immune Complex-Mediated Membranoproliferative glomerulonephritis (IC-MPGN) are rare diseases characterized by glomerular deposition of C3 caused by dysregulation of the alternative pathway (AP) of complement.
Rossella Piras +12 more
doaj +1 more source
Factor H family proteins and human diseases [PDF]
Complement is a major defense system of innate immunity and aimed to destroy microbes. One of the central complement regulators is factor H, which belongs to a protein family that includes CFHL1 and five factor H-related (CFHR) proteins.
Peter F. Zipfel +3 more
core +1 more source
Mechanism of Borrelia immune evasion by FhbA-related proteins.
Immune evasion facilitates survival of Borrelia, leading to infections like relapsing fever and Lyme disease. Important mechanism for complement evasion is acquisition of the main host complement inhibitor, factor H (FH). By determining the 2.2 Å crystal
Konstantin Kogan +6 more
doaj +1 more source
Factor H‐related protein 1: a complement regulatory protein and guardian of necrotic‐type surfaces [PDF]
Factor H‐related protein 1 (FHR‐1) is a member of the factor H protein family, which is involved in regulating innate immune complement reactions. Genetic modification of the encoding gene, CFHR1 on human chromosome 1, is involved in diseases such as age‐related macular degeneration, C3 glomerulopathy and atypical haemolytic uraemic syndrome ...
Skerka, Christine +5 more
openaire +3 more sources
Factor H-related protein 1 (FHR-1) is associated with atherosclerotic cardiovascular disease [PDF]
AbstractAtherosclerotic cardiovascular disease (ACVD) is a lipid-driven inflammatory disease and one of the leading causes of death worldwide. Lipid deposits in the arterial wall lead to the formation of plaques that involve lipid oxidation, cellular necrosis, and complement activation, resulting in inflammation and thrombosis.
Irmscher, S. +23 more
openaire +4 more sources
Background and purposesRecent developments in high-throughput proteomic approach have shown the potential to discover biomarkers for diagnosing acute stroke and to elucidate the pathomechanisms specific to different stroke subtypes. We aimed to determine
Shubham Misra +12 more
doaj +1 more source
Borrelia recurrentis employs a novel multifunctional surface protein with anti-complement, anti-opsonic and invasive potential to escape innate immunity [PDF]
Borrelia recurrentis, the etiologic agent of louse-borne relapsing fever in humans, has evolved strategies, including antigenic variation, to evade immune defence, thereby causing severe diseases with high mortality rates.
Schott, Melanie +34 more
core +2 more sources
Regulation of regulators: Role of the complement factor H-related proteins
The complement system, while being an essential and very efficient effector component of innate immunity, may cause damage to the host and result in various inflammatory, autoimmune and infectious diseases or cancer, when it is improperly activated or regulated.
Cserhalmi, Marcell +4 more
openaire +4 more sources
Human complement factor H. Two factor H proteins are derived from alternatively spliced transcripts [PDF]
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 ...
Cornelia Estaller +7 more
core +1 more source
Complement factor H family proteins in their non-canonical role as modulators of cellular functions [PDF]
Complement factor H is a major regulator of the alternative pathway of the complement system. The factor H-related proteins are less characterized, but recent data indicate that they rather promote complement activation.
Noémi Sándor +7 more
core +1 more source

