MUC-2 mucin production in Hirschsprung's disease: Possible association with enterocolitis development

• Published in: Journal of pediatric surgery 2003-03, Vol.38 (3), p.417-421
• Main Authors: Mattar, Aladdein F., Coran, Arnold G., Teitelbaum, Daniel H.
• Format: Article
• Language: English
• Subjects: Bacterial Translocation; Case-Control Studies; Diarrhea - metabolism; Diarrhea - virology; enterocolitis; Enterocolitis - etiology; Feces - chemistry; Female; Hirschsprung Disease - complications; Hirschsprung Disease - metabolism; Hirschsprung's disease; Humans; Infant; Male; mucin genes; Mucin-2; Mucins - analysis; Mucins - deficiency; Mucins - physiology
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1053/jpsu.2003.50071

Background/Purpose: The etiology of Hirschsprung's-associated enterocolitis (HAEC) is unknown. Previous investigations have suggested that abnormal production of mucins may have an etiologic role. Recently, a series of mucin genes have been identified. MUC-2 is the predominant mucin expressed in humans. The authors have shown previously in vitro that use of MUC-2 can prevent bacterial translocation. Based on this, it was hypothesized that those patients with Hirschsprung's disease (HD) would have an abnormal production of MUC-2 compared with normal patients. Methods: Fresh stool specimens were collected from children with a diagnosis of HD (with or without HAEC) and from age-matched control patients. Protein was extracted, and MUC-2 was detected with Western blot analysis. MUC-2 protein expression was quantified by densitometry measurements. Results are expressed as mean density ± SD. Statistical comparison was done with unpaired t tests, with P less than.05 being considered significant. Results: MUC-2 expression was detected in all control patients (mean density, 121 ± 47). MUC-2 level was lowest in one child with a viral-induced diarrhea (density = 71). In those patients with HD, levels of MUC-2 protein expression were significantly lower (P