Serum complement factor I is associated with disease activity of systemic lupus erythematosus. [PDF]
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited.
Tseng MH+7 more
europepmc +2 more sources
Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome. [PDF]
Atypical hemolytic uremic syndrome (aHUS) is associated with defective complement regulation. Disease-associated mutations have been described in the genes encoding the complement regulators complement factor H, membrane cofactor protein, factor B, and ...
Peter F Zipfel+13 more
doaj +5 more sources
A surface lipoprotein on Pasteurella multocida binds complement factor I to promote immune evasion. [PDF]
Pasteurella multocida is the leading cause of wound infections in humans following animals' bites or scratches. This bacterium is also commonly found in the respiratory tract of many mammals and can cause serious diseases resulting in the rapid death of ...
Quynh Huong Nguyen+7 more
doaj +2 more sources
C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report [PDF]
Background It has been suggested that C3 glomerulonephritis (C3GN) and atypical hemolytic-uremic syndrome (a stereotypical phenotype of thrombotic microangiopathy), two rare entities caused by complement alternative pathway dysregulation share ...
Jiqiu Wen+5 more
doaj +2 more sources
Atypical hemolytic-uremic syndrome due to complement factor I mutation. [PDF]
Atypical hemolytic-uremic syndrome (aHUS) is a rare disease of complement dysregulation leading to thrombotic microangiopathy (TMA). Renal involvement and progression to end-stage renal disease are common in untreated patients. We report a 52-year-old female patient who presented with severe acute kidney injury, microangiopathic hemolytic anemia, and ...
Almalki AH+5 more
europepmc +5 more sources
Plasma Levels of Complement Factor I and C4b Peptides Are Associated with HIV Suppression. [PDF]
Individuals who exhibit long-term HIV suppression and CD4 T-cell preservation without antiretroviral therapy are of great interest for HIV research. There is currently no robust method for rapid identification of these “HIV controller” subjects; however,
Wu B+10 more
europepmc +2 more sources
The Staphylococcus aureus protein Sbi acts as a complement inhibitor and forms a tripartite complex with host complement Factor H and C3b. [PDF]
The Gram-positive bacterium Staphylococcus aureus, similar to other pathogens, binds human complement regulators Factor H and Factor H related protein 1 (FHR-1) from human serum.
Katrin Haupt+7 more
doaj +6 more sources
The molecular basis of hereditary complement factor I deficiency. [PDF]
The molecular basis of hereditary complement factor I deficiency is described in two pedigrees. In one pedigree, there were two factor I-deficient siblings, one of whom was asymptomatic and the other suffered from recurrent pyogenic infections.
T. Vyse+9 more
semanticscholar +4 more sources
Complete recovery after complement factor I deficiency associated fulminant acute hemorrhagic leukoencephalitis: a case report [PDF]
IntroductionAcute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant neuroinflammatory disease with high mortality rate. It most often occurs after infections; however, the exact etiology of the disease remains unclear.
Fanni Szumutku+12 more
doaj +2 more sources
Complement factor I: Regulatory nexus, driver of immunopathology, and therapeutic.
Complement factor I (FI) is the nexus for classical, lectin and alternative pathway complement regulation. FI is an 88 kDa plasma protein that circulates in an inactive configuration until it forms a trimolecular complex with its cofactor and substrate whereupon a structural reorganization allows the catalytic triad to cleave its substrates, C3b and ...
T. Hallam+3 more
semanticscholar +4 more sources