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 language | English |
|---|---|
| Pages (from-to) | 350-357 |
| Number of pages | 8 |
| Journal | Gastroenterology |
| Volume | 121 |
| Issue number | 2 |
| Publication status | Published - 2001 |
| Externally published | Yes |
Fingerprint
ASJC Scopus subject areas
- Gastroenterology
Cite this
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 journal › Article
}
TY - JOUR
T1 - Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the α3 nicotinic acetylcholine receptor subunit
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
AU - Thomas, Gareth A O
AU - Smith, Virpi
PY - 2001
Y1 - 2001
N2 - 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.
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.
UR - http://www.scopus.com/inward/record.url?scp=0034909750&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034909750&partnerID=8YFLogxK
M3 - Article
VL - 121
SP - 350
EP - 357
JO - Gastroenterology
JF - Gastroenterology
SN - 0016-5085
IS - 2
ER -