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 …

Complement activation, regulation, and molecular basis for complement‐related diseases

G Bajic, SE Degn, S Thiel, GR Andersen - The EMBO journal, 2015 - embopress.org
The complement system is an essential element of the innate immune response that
becomes activated upon recognition of molecular patterns associated with microorganisms …

Atypical aHUS: state of the art

CM Nester, T Barbour, SR de Cordoba… - Molecular …, 2015 - Elsevier
Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs)
have revealed distinct disease mechanisms within this heterogeneous group of diseases. As …

[HTML][HTML] Atypical hemolytic uremic syndrome

D Kavanagh, TH Goodship, A Richards - Seminars in nephrology, 2013 - Elsevier
Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia,
thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease …

Complement control protein factor H: the good, the bad, and the inadequate

VP Ferreira, MK Pangburn, C Cortés - Molecular immunology, 2010 - Elsevier
The complement system is an essential component of the innate immune system that
participates in elimination of pathogens and altered host cells and comprises an essential …

Protection of host cells by complement regulators

CQ Schmidt, JD Lambris, D Ricklin - Immunological reviews, 2016 - Wiley Online Library
The complement cascade is an ancient immune‐surveillance system that not only provides
protection from pathogen invasion but has also evolved to participate in physiological …

Statistical validation of rare complement variants provides insights into the molecular basis of atypical hemolytic uremic syndrome and C3 glomerulopathy

AJ Osborne, M Breno, NG Borsa, F Bu… - The Journal of …, 2018 - journals.aai.org
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated
with dysregulation and overactivation of the complement alternative pathway. Typically …

Complement factor H in host defense and immune evasion

R Parente, SJ Clark, A Inforzato, AJ Day - Cellular and Molecular Life …, 2017 - Springer
Complement is the major humoral component of the innate immune system. It recognizes
pathogen-and damage-associated molecular patterns, and initiates the immune response in …

Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical …

I Moore, L Strain, I Pappworth… - Blood, The Journal …, 2010 - ashpublications.org
Factor H autoantibodies have been reported in approximately 10% of patients with atypical
hemolytic uremic syndrome (aHUS) and are associated with deficiency of factor H–related …

Structure of complement fragment C3b–factor H and implications for host protection by complement regulators

J Wu, YQ Wu, D Ricklin, BJC Janssen, JD Lambris… - Nature …, 2009 - nature.com
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 …