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• W2991378049 abstract "Purpose The pathophysiology of Hirschsprung's associated enterocolitis (HAEC) is not understood. Abnormal intestinal motility and altered intestinal epithelial barrier function have been suggested to play a key role in the causation of HAEC. Protease-activated receptors (PARs) 1 and 2, have been implicated in inflammatory reactions, intestinal permeability and modulation of motility in the gut. Methods We investigated PAR-1 and PAR-2 protein expression in aganglionic and ganglionic regions of patients with Hirschsprung's Disease (HSCR) (n = 10) versus normal control colon (n = 10). Protein distribution was assessed by using immunofluorescence and confocal microscopy. Gene and protein expression were quantified using quantitative real-time polymerase chain reaction (qPCR), western blot analysis, and densitometry. Results qPCR and Western blot analysis revealed that PAR-1 and PAR-2 expression was significantly increased in ganglionic and aganglionic bowel in HSCR compared to controls (p < 0.003). Confocal microscopy revealed strong PAR-1 and PAR-2 expression in smooth muscles, interstitial cells of Cajal (ICCs), platelet-derived growth factor-alpha receptor-positive (PDGFRα+) cells, enteric neurons and epithelium in the ganglionic and aganglionic bowel compared to controls. Conclusion Increased PAR-1 and PAR-2 expression in the colon of patients with HSCR suggests that excessive local release of PAR activating proteases may trigger inflammatory responses leading to HAEC." @default.
• W2991378049 created "2019-12-05" @default.
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• W2991378049 date "2020-08-01" @default.
• W2991378049 modified "2023-10-14" @default.
• W2991378049 title "Increased protease activated receptors in the colon of patients with Hirschsprung's disease" @default.
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• W2991378049 doi "https://doi.org/10.1016/j.jpedsurg.2019.11.009" @default.
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