Complement system part I–molecular mechanisms of activation and regulation
NS Merle, SE Church, V Fremeaux-Bacchi… - Frontiers in …, 2015 - frontiersin.org
Complement is a complex innate immune surveillance system, playing a key role in defense
against pathogens and in host homeostasis. The complement system is initiated by …
against pathogens and in host homeostasis. The complement system is initiated by …
[HTML][HTML] Overview of complement activation and regulation
Complement is an important component of the innate immune system that is crucial for
defense from microbial infections and for clearance of immune complexes and injured cells …
defense from microbial infections and for clearance of immune complexes and injured cells …
Atypical hemolytic–uremic syndrome
The hemolytic–uremic syndrome, which is characterized by nonimmune hemolytic anemia,
thrombocytopenia, and renal impairment, occurs most frequently in young children. Most …
thrombocytopenia, and renal impairment, occurs most frequently in young children. Most …
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome
J Caprioli, M Noris, S Brioschi, G Pianetti, F Castelletti… - Blood, 2006 - ashpublications.org
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy with manifestations of
hemolytic anemia, thrombocytopenia, and renal impairment. Genetic studies have shown …
hemolytic anemia, thrombocytopenia, and renal impairment. Genetic studies have shown …
Activation of human CD4+ cells with CD3 and CD46 induces a T-regulatory cell 1 phenotype
The immune system must distinguish not only between self and non-self, but also between
innocuous and pathological foreign antigens to prevent unnecessary or self-destructive …
innocuous and pathological foreign antigens to prevent unnecessary or self-destructive …
Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome
V Frémeaux-Bacchi, EC Miller… - Blood, The Journal …, 2008 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation. In
approximately 50% of patients, mutations have been described in the genes encoding the …
approximately 50% of patients, mutations have been described in the genes encoding the …
Structure of complement fragment C3b–factor H and implications for host protection by complement regulators
Factor H (FH) is an abundant regulator of complement activation and protects host cells from
self-attack by complement. Here we provide insight into the regulatory activity of FH by …
self-attack by complement. Here we provide insight into the regulatory activity of FH by …
Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort
JE Salmon, C Heuser, M Triebwasser… - PLoS …, 2011 - journals.plos.org
Background Pregnancy in women with systemic lupus erythematosus (SLE) or
antiphospholipid antibodies (APL Ab)—autoimmune conditions characterized by …
antiphospholipid antibodies (APL Ab)—autoimmune conditions characterized by …
Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome
A Richards, EJ Kemp, MK Liszewski… - Proceedings of the …, 2003 - National Acad Sciences
Membrane cofactor protein (MCP; CD46) is a widely expressed transmembrane
complement regulator. Like factor H it inhibits complement activation by regulating C3b …
complement regulator. Like factor H it inhibits complement activation by regulating C3b …