Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha 3 nicotinic acetylcholine receptor subunit

C E Richardson, J M Morgan, B Jasani, J T Green, J Rhodes, G T Williams, J Lindstrom, S Wonnacott, G A O Thomas, V Smith

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Abstract

Background & Aims: The megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare disease of childhood that presents early with intestinal hypoperistalsis, hydronephrosis, and hydroureters. Transgenic mice that lack the alpha3 subunit containing nicotinic acetylcholine (nAChR) have a phenotype similar to that of MMIHS. Methods: We examined the expression of this subunit in control and MMIHS tissue derived from patients using in situ hybridization (ISH) and immunocytochemistry (ICC). Results: In controls, both techniques showed a wide distribution of alpha3 nAChRs present in ganglion cells, muscle, and epithelium. By contrast, most MMIHS tissue gave negative staining with ISH and variable results with ICC. Conclusions: These observations are consistent with a lack of alpha3 nAChRs contributing to the pathogenesis of MMIHS.
Original languageEnglish
Pages (from-to)350-357
Number of pages8
JournalGastroenterology
Volume121
Issue number2
Publication statusPublished - 2001

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    Richardson, C. E., Morgan, J. M., Jasani, B., Green, J. T., Rhodes, J., Williams, G. T., Lindstrom, J., Wonnacott, S., Thomas, G. A. O., & Smith, V. (2001). Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha 3 nicotinic acetylcholine receptor subunit. Gastroenterology, 121(2), 350-357.