Aladdein F Mattar1, Arnold G Coran, Daniel H Teitelbaum. 1. Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan 48109, USA.
Abstract
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 <.05) than controls (12 +/- 15 for all HD patients). Levels of MUC-2 were lowest (nondetectable) in 2 HD patients who had clinical evidence of HAEC. CONCLUSIONS: MUC-2 production is markedly depressed in patients with Hirschsprung's disease and is absent with enterocolitis. This decline in protein expression may result in a decrease in epithelial barrier function and be a predisposing factor in the development of HAEC. Copyright 2003, Elsevier Science (USA). All rights reserved.
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 <.05) than controls (12 +/- 15 for all HDpatients). Levels of MUC-2 were lowest (nondetectable) in 2 HDpatients who had clinical evidence of HAEC. CONCLUSIONS:MUC-2 production is markedly depressed in patients with Hirschsprung's disease and is absent with enterocolitis. This decline in protein expression may result in a decrease in epithelial barrier function and be a predisposing factor in the development of HAEC. Copyright 2003, Elsevier Science (USA). All rights reserved.
Authors: Sureyya Ozcan; Hyun Joo An; Ana C Vieira; Gun Wook Park; Jae Han Kim; Mark J Mannis; Carlito B Lebrilla Journal: J Proteome Res Date: 2013-02-06 Impact factor: 4.466
Authors: Ming Li; Dorothy L Patton; Yvonne Cosgrove-Sweeney; Deena Ratner; Lisa C Rohan; Alexander M Cole; Patrick M Tarwater; Phalguni Gupta; Bharat Ramratnam Journal: J Acquir Immune Defic Syndr Date: 2011-12-01 Impact factor: 3.731
Authors: Joseph F Pierre; Amanda J Barlow-Anacker; Christopher S Erickson; Aaron F Heneghan; Glen E Leverson; Scot E Dowd; Miles L Epstein; Kenneth A Kudsk; Ankush Gosain Journal: J Pediatr Surg Date: 2014-08 Impact factor: 2.545