Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit

Charles E. Richardson, John M. Morgan, Bharat Jasani, John T. Green, John Rhodes, Geraint T. Williams, Jon Lindstrom, Sue Wonnacott, Gareth A O Thomas, Virpi Smith

Research output: Contribution to journalArticle

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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 α3 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 immunocyto-chemistry (ICC). Results: In controls, both techniques showed a wide distribution of α3 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 α3 nAChRs contributing to the pathogenesis of MMIHS.

Original languageEnglish
Pages (from-to)350-357
Number of pages8
JournalGastroenterology
Volume121
Issue number2
Publication statusPublished - 2001
Externally publishedYes

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Nicotinic Receptors
In Situ Hybridization
Negative Staining
Hydronephrosis
Rare Diseases
Ganglia
Muscle Cells
Transgenic Mice
Acetylcholine
Epithelium
Megacystis microcolon intestinal hypoperistalsis syndrome
Phenotype

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Richardson, C. E., Morgan, J. M., Jasani, B., Green, J. T., Rhodes, J., Williams, G. T., ... Smith, V. (2001). Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit. Gastroenterology, 121(2), 350-357.

Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit. / Richardson, Charles E.; Morgan, John M.; Jasani, Bharat; Green, John T.; Rhodes, John; Williams, Geraint T.; Lindstrom, Jon; Wonnacott, Sue; Thomas, Gareth A O; Smith, Virpi.

In: Gastroenterology, Vol. 121, No. 2, 2001, p. 350-357.

Research output: Contribution to journalArticle

Richardson, CE, Morgan, JM, Jasani, B, Green, JT, Rhodes, J, Williams, GT, Lindstrom, J, Wonnacott, S, Thomas, GAO & Smith, V 2001, 'Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit', Gastroenterology, vol. 121, no. 2, pp. 350-357.
Richardson CE, Morgan JM, Jasani B, Green JT, Rhodes J, Williams GT et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit. Gastroenterology. 2001;121(2):350-357.
Richardson, Charles E. ; Morgan, John M. ; Jasani, Bharat ; Green, John T. ; Rhodes, John ; Williams, Geraint T. ; Lindstrom, Jon ; Wonnacott, Sue ; Thomas, Gareth A O ; Smith, Virpi. / Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit. In: Gastroenterology. 2001 ; Vol. 121, No. 2. pp. 350-357.
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AU - Richardson, Charles E.

AU - Morgan, John M.

AU - Jasani, Bharat

AU - Green, John T.

AU - Rhodes, John

AU - Williams, Geraint T.

AU - Lindstrom, Jon

AU - Wonnacott, Sue

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AB - 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 α3 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 immunocyto-chemistry (ICC). Results: In controls, both techniques showed a wide distribution of α3 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 α3 nAChRs contributing to the pathogenesis of MMIHS.

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