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

Research output: Contribution to journalArticle

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.
LanguageEnglish
Pages350-357
Number of pages8
JournalGastroenterology
Volume121
Issue number2
StatusPublished - 2001

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

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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 alpha 3 nicotinic acetylcholine receptor subunit. Gastroenterology, 121(2), 350-357.

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

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 alpha 3 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 alpha 3 nicotinic acetylcholine receptor subunit. Gastroenterology. 2001;121(2):350-357.
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. / Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha 3 nicotinic acetylcholine receptor subunit. In: Gastroenterology. 2001 ; Vol. 121, No. 2. pp. 350-357.
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AU - Richardson, C E

AU - Morgan, J M

AU - Jasani, B

AU - Green, J T

AU - Rhodes, J

AU - Williams, G T

AU - Lindstrom, J

AU - Wonnacott, S

AU - Thomas, G A O

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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 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.

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