Altered expression of caveolin-1 in the colon of patients with Hirschsprung’s disease
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In brief
In brief
Study demonstrates significantly decreased caveolin-1 expression in both aganglionic and ganglionic colon segments of Hirschsprung's disease patients compared to controls. Reduced caveolin-1 may dysregulate nitric oxide synthase function and compromise intestinal barrier integrity, potentially explaining increased susceptibility to Hirschsprung-associated enterocolitis.
Written by the GCMD Library team from the article.
Abstract
Background/purpose
The pathogenesis of Hirschsprung’s disease-associated enterocolitis (HAEC) is unclear. Caveolin-1 (Cav-1) regulates the functions of different nitric oxide synthase (NOS) isoforms, which play critical roles in inflammation and intestinal epithelial barrier function. We designed this study to investigate the hypothesis that Cav-1 expression is altered in the bowel of patients with Hirschsprung’s disease (HSCR).
Methods
HSCR tissue specimens (n = 10) were collected at the time of pull-through surgery and control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 10). qRT-PCR analysis was undertaken to quantify Cav-1 gene expression, and Western blot analysis was undertaken to determine Cav-1 protein quantification. Immunolabelling of Cav-1 proteins was visualized using confocal microscopy.
Results
qRT-PCR and Western blot analysis revealed that Cav-1 was significantly downregulated in the aganglionic and ganglionic colon of patients with HSCR compared to controls (p < 0.01). Confocal microscopy revealed a markedly decreased expression of Cav-1 in colonic epithelium of aganglionic and ganglionic bowel of patients with HSCR compared to controls.
Conclusion
To our knowledge, this is the first report of significantly decreased Cav-1 expression in patients with HSCR. Decreased expression of Cav-1 in the bowel of HSCR may increase susceptibility to HAEC in HSCR.