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Gastroenterology. 2001 Aug;121(2):350-7.

Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha3 nicotinic acetylcholine receptor subunit.

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  • 1Department of Gastroenterology, University Hospital of Wales, Heath Park, Cardiff, Wales CF14 4XW, UK.

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.

PMID:
11487544
[PubMed - indexed for MEDLINE]
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