Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit
CE Richardson, JM Morgan, B Jasani, JT Green… - Gastroenterology, 2001 - Elsevier
CE Richardson, JM Morgan, B Jasani, JT Green, J Rhodes, GT Williams, J Lindstrom…
Gastroenterology, 2001•ElsevierBackground & 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 immunocytochemistry (ICC). Results: In controls, both …
(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 immunocytochemistry (ICC). Results: In controls, both …
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 immunocytochemistry (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.
Elsevier
