Increased Act1/IL-17R expression in Hirschsprung's disease.
Pediatr Surg Int
; 32(12): 1201-1207, 2016 Dec.
Article
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| MEDLINE
| ID: mdl-27660002
ABSTRACT
PURPOSE:
Hirschsprung's disease -associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Altered intestinal epithelial barrier function is implicated in the pathogenesis of HAEC. IL-17 is a proinflammatory cytokine that plays a crucial role in host defense against microbial organisms in the development of inflammatory diseases. Act1 is an essential adaptor molecule required for the IL-17-mediated inflammatory responses via interaction with IL-17 receptor (IL-17R). We designed this study to investigate the hypothesis that Act1/Il-17R expression is upregulated in HSCR.METHODS:
We investigated Act1 and IL17R expression in ganglionic andaganglionic bowel of HD patients (n = 10) and controls (n = 10). qPCR, Western blotting and confocal immunofluorescence were performed. MAINRESULTS:
qPCR and Western blot analysis revealed that Act1 and IL17R are strongly expressed in the aganglionic and ganglionic colon of patients with HSCR. Act1 and IL17R expression was significantly increased in HSCR specimens compared to controls (p < 0.05). Confocal microscopy revealed a markedly increased expression of Act1 and IL17R in the colonic epithelium of patients with HSCR compared to controls.CONCLUSION:
To our knowledge, we report, for the first time, the expression of Act1 in the human colon. The increased expression of Act1 and Il-17 in the aganglionic and ganglionic bowel in HSCR may result in IL-17-mediated increased inflammatory response leading to the development of HAEC.Palabras clave
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Interleucina-17
/
Péptidos y Proteínas Asociados a Receptores de Factores de Necrosis Tumoral
/
Enfermedad de Hirschsprung
Límite:
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Pediatr Surg Int
Asunto de la revista:
PEDIATRIA
Año:
2016
Tipo del documento:
Article
País de afiliación:
Irlanda