FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2297386413> ?p ?o ?g. }

• W2297386413 endingPage "444" @default.
• W2297386413 startingPage "433" @default.
• W2297386413 abstract "Abstract Papillon-Lefévre syndrome is a rare, inherited, autosomal-recessive disease, characterized by palmoplantar keratosis and severe prepubertal periodontitis, leading to premature loss of all teeth. Papillon-Lefévre syndrome is caused by a mutation in the cathepsin C gene, resulting in complete loss of activity and subsequent failure to activate immune response proteins. Periodontitis in Papillon-Lefévre syndrome is thought to arise from failure to eliminate periodontal pathogens as a result of cathepsin C deficiency, although mechanistic pathways remain to be elucidated. The aim of this study was to characterize comprehensively neutrophil function in Papillon-Lefévre syndrome. Peripheral blood neutrophils were isolated from 5 patients with Papillon-Lefévre syndrome, alongside matched healthy control subjects. For directional chemotactic accuracy, neutrophils were exposed to the chemoattractants MIP-1α and fMLP and tracked by real-time videomicroscopy. Reactive oxygen species generation was measured by chemiluminescence. Neutrophil extracellular trap formation was assayed fluorometrically, and proinflammatory cytokine release was measured following overnight culture of neutrophils with relevant stimuli. Neutrophil serine protease deficiencies resulted in a reduced ability of neutrophils to chemotax efficiently and an inability to generate neutrophil extracellular traps. Neutrophil extracellular trap-bound proteins were also absent in Papillon-Lefévre syndrome, and Papillon-Lefévre syndrome neutrophils released higher levels of proinflammatory cytokines in unstimulated and stimulated conditions, and plasma cytokines were elevated. Notably, neutrophil chemoattractants MIP-1α and CXCL8 were elevated in Papillon-Lefévre syndrome neutrophils, as was reactive oxygen species formation. We propose that relentless recruitment and accumulation of hyperactive/reactive neutrophils (cytokines, reactive oxygen species) with increased tissue transit times into periodontal tissues, alongside a reduced antimicrobial capacity, create a locally destructive chronic inflammatory cycle in Papillon-Lefévre syndrome." @default.
• W2297386413 created "2016-06-24" @default.
• W2297386413 creator A5006587597 @default.
• W2297386413 creator A5012697008 @default.
• W2297386413 creator A5037623808 @default.
• W2297386413 creator A5040791868 @default.
• W2297386413 creator A5051000545 @default.
• W2297386413 creator A5051234795 @default.
• W2297386413 creator A5070092970 @default.
• W2297386413 creator A5080971750 @default.
• W2297386413 date "2016-03-08" @default.
• W2297386413 modified "2023-10-12" @default.
• W2297386413 title "Characterization of neutrophil function in Papillon-Lefèvre syndrome" @default.
• W2297386413 cites W1525631450 @default.
• W2297386413 cites W1535189536 @default.
• W2297386413 cites W1544295266 @default.
• W2297386413 cites W1576707839 @default.
• W2297386413 cites W1589237240 @default.
• W2297386413 cites W1598588804 @default.
• W2297386413 cites W1679127384 @default.
• W2297386413 cites W1762827693 @default.
• W2297386413 cites W1873474378 @default.
• W2297386413 cites W1950981769 @default.
• W2297386413 cites W1982268827 @default.
• W2297386413 cites W2001607419 @default.
• W2297386413 cites W2003310710 @default.
• W2297386413 cites W2008913472 @default.
• W2297386413 cites W2020022405 @default.
• W2297386413 cites W2033220681 @default.
• W2297386413 cites W2033405833 @default.
• W2297386413 cites W2035721892 @default.
• W2297386413 cites W2048209972 @default.
• W2297386413 cites W2051245226 @default.
• W2297386413 cites W2057295828 @default.
• W2297386413 cites W2062752766 @default.
• W2297386413 cites W2063409764 @default.
• W2297386413 cites W2064820872 @default.
• W2297386413 cites W2067485115 @default.
• W2297386413 cites W2067769931 @default.
• W2297386413 cites W2068724980 @default.
• W2297386413 cites W2084259901 @default.
• W2297386413 cites W2104934425 @default.
• W2297386413 cites W2110602760 @default.
• W2297386413 cites W2114048811 @default.
• W2297386413 cites W2115959782 @default.
• W2297386413 cites W2116558722 @default.
• W2297386413 cites W2125894404 @default.
• W2297386413 cites W2129762153 @default.
• W2297386413 cites W2140626436 @default.
• W2297386413 cites W2144142375 @default.
• W2297386413 cites W2154702360 @default.
• W2297386413 cites W2158887211 @default.
• W2297386413 cites W2165493879 @default.
• W2297386413 cites W2170872843 @default.
• W2297386413 cites W2171697344 @default.
• W2297386413 cites W2172127117 @default.
• W2297386413 cites W2183333696 @default.
• W2297386413 cites W2246380774 @default.
• W2297386413 cites W2260018086 @default.
• W2297386413 cites W2273883970 @default.
• W2297386413 cites W2337701902 @default.
• W2297386413 cites W2582752143 @default.
• W2297386413 cites W30364595 @default.
• W2297386413 doi "https://doi.org/10.1189/jlb.5a1015-489r" @default.
• W2297386413 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/26957212" @default.
• W2297386413 hasPublicationYear "2016" @default.
• W2297386413 type Work @default.
• W2297386413 sameAs 2297386413 @default.
• W2297386413 citedByCount "69" @default.
• W2297386413 countsByYear W22973864132016 @default.
• W2297386413 countsByYear W22973864132017 @default.
• W2297386413 countsByYear W22973864132018 @default.
• W2297386413 countsByYear W22973864132019 @default.
• W2297386413 countsByYear W22973864132020 @default.
• W2297386413 countsByYear W22973864132021 @default.
• W2297386413 countsByYear W22973864132022 @default.
• W2297386413 countsByYear W22973864132023 @default.
• W2297386413 crossrefType "journal-article" @default.
• W2297386413 hasAuthorship W2297386413A5006587597 @default.
• W2297386413 hasAuthorship W2297386413A5012697008 @default.
• W2297386413 hasAuthorship W2297386413A5037623808 @default.
• W2297386413 hasAuthorship W2297386413A5040791868 @default.
• W2297386413 hasAuthorship W2297386413A5051000545 @default.
• W2297386413 hasAuthorship W2297386413A5051234795 @default.
• W2297386413 hasAuthorship W2297386413A5070092970 @default.
• W2297386413 hasAuthorship W2297386413A5080971750 @default.
• W2297386413 hasBestOaLocation W22973864131 @default.
• W2297386413 hasConcept C134897094 @default.
• W2297386413 hasConcept C164027704 @default.
• W2297386413 hasConcept C170493617 @default.
• W2297386413 hasConcept C181199279 @default.
• W2297386413 hasConcept C182220744 @default.
• W2297386413 hasConcept C203014093 @default.
• W2297386413 hasConcept C2776914184 @default.
• W2297386413 hasConcept C2777132095 @default.
• W2297386413 hasConcept C54166955 @default.
• W2297386413 hasConcept C55493867 @default.
• W2297386413 hasConcept C86803240 @default.

• next