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UnknownLow-Avidity Autoantibodies against Bactericidal/Permeability-Increasing Protein Occur in Gram-Negative and Gram-Positive Bacteremia.
Antibody autoreactivity against bactericidal/permeability-increasing protein (BPI) is strongly associated with Pseudomonas aeruginosa infection in cystic fibrosis (CF), non-CF bronchiectasis (BE), and chronic obstructive pulmonary disease (COPD). We examined the pathogen-specific nature of this auto...
S-EPMC7504969 | biostudies-literature
UnknownKilling three birds with one BPI: Bactericidal, opsonic, and anti-inflammatory functions.
Bactericidal/permeability-increasing protein (BPI) is an anti-microbial protein predominantly expressed in azurophilic granules of neutrophils. BPI has been shown to mediate cytocidal and opsonic activity against Gram-negative bacteria, while also blunting inflammatory activity of lipopolysaccharide...
S-EPMC8187252 | biostudies-literature
UnknownDifferential Enhancement of Neutrophil Phagocytosis by Anti-Bactericidal/Permeability-Increasing Protein Antibodies.
Bactericidal/permeability-increasing protein (BPI) plays a major role in innate immunity through the ability of the N-terminal domain (NTD) to bind LPS, mediate cytotoxicity, and block LPS-induced inflammation. The C-terminal domain mediates phagocytosis of bacteria bound to the NTD. These two domai...
S-EPMC8354091 | biostudies-literature
UnknownCystic fibrosis-related kidney disease-emerging morbidity and disease modifier.
Cystic fibrosis (CF) is a life-shortening multisystem disease resulting from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, causing the most devastating phenotypes in the airway and pancreas. Significant advances in treatment for CF lung disease, including the expa...
S-EPMC12549735 | biostudies-literature
UnknownDissociation of systemic and mucosal autoimmunity in cystic fibrosis.

Background

Pseudomonas aeruginosa accounts for ~80% of cystic fibrosis (CF) airway infection. It shows a remarkable correlation with presence of autoantibody to bactericidal/permeability-increasing protein (BPI), which is not understood. In this study, we sought to better understand the char...
S-EPMC10377741 | biostudies-literature
UnknownAcute skin exposure to ultraviolet light triggers neutrophil-mediated kidney inflammation.
Photosensitivity to ultraviolet (UV) light affects up to ∼80% of lupus patients. Sunlight exposure can exacerbate local as well as systemic manifestations of lupus, including nephritis, by mechanisms that are poorly understood. Here, we report that acute skin exposure to UV light triggers a neutroph...
S-EPMC7826360 | biostudies-literature
UnknownRegulation of Pseudomonas aeruginosa-Mediated Neutrophil Extracellular Traps.
Pseudomonas aeruginosa is the most prevalent opportunistic pathogen in the airways of cystic fibrosis (CF) patients. The pulmonary disorder is characterized by recurrent microbial infections and an exaggerated host inflammatory immune response led primarily by influx of neutrophils. Under the...
S-EPMC6657737 | biostudies-literature
UnknownComplement Deficiencies Result in Surrogate Pathways of Complement Activation in Novel Polygenic Lupus-like Models of Kidney Injury.
Lupus nephritis (LN) is a major contributor to morbidity and mortality in lupus patients, but the mechanisms of kidney damage remain unclear. In this study, we introduce, to our knowledge, novel models of LN designed to resemble the polygenic nature of human lupus by embodying three key genetic alte...
S-EPMC7365257 | biostudies-literature
UnknownThe early local and systemic Type I interferon responses to ultraviolet B light exposure are cGAS dependent.
Most systemic lupus erythematosus (SLE) patients are photosensitive and ultraviolet B light (UVB) exposure worsens cutaneous disease and precipitates systemic flares of disease. The pathogenic link between skin disease and systemic exacerbations in SLE remains elusive. In an acute model of UVB-trigg...
S-EPMC7220927 | biostudies-literature
UnknownThe role of VISTA engagement in limiting neutrophil-mediated inflammation.
A growing body of evidence suggests that the immune checkpoint inhibitory receptor VISTA plays a central role in the regulation of innate immunity in the settings of inflammatory diseases and cancer. Neutrophils are among the cells that have the highest membrane density of surface VISTA. In this stu...
S-EPMC12336919 | biostudies-literature
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