Analysis Tools
renal/urinary system
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• failure of urine emptying into the bladder
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• some mice exhibit hydronephrosis due to the failure of urine emptying into the bladder
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• 2-fold decrease in the concentration of dense bodies in the bladder
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• mice show a defect in dense bodies of bladder detrusor SMCs
• the length of cytoskeletal dense bodies in longitudinally oriented SMCs is nearly 3x greater than in wild-type mice
• early onset thinning of detrusor muscle of the bladder, with postnatal mice showing thinning and compaction of the visceral smooth muscle layers
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• postnatal mice show loss of the transitional epithelium of the bladder
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• mice exhibit distention of the bladder beginning at E18.5
• in some cases, the developing bladder expands as much as to encroach upon the abdominal cavity
• 100% of mice show enlargement of the urinary bladder after parturition and the first feeding
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muscle
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• postnatal mice exhibit thinning and compaction of the visceral smooth muscle layers in the stomach
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• mice show a defect in dense bodies of bladder detrusor SMCs
• the length of cytoskeletal dense bodies in longitudinally oriented SMCs is nearly 3x greater than in wild-type mice
• early onset thinning of detrusor muscle of the bladder, with postnatal mice showing thinning and compaction of the visceral smooth muscle layers
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• the outer longitudinal layer of the intestine shows an increase in Ki67 staining of smooth muscle cells (SMCs) but no obvious signs of cell death as measured by TUNEL staining is seen
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• more than 3-fold decrease in KCL- or carbachol-induced contractility in intestinal ring segments indicating intestinal hypoperistalsis
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• mice show a notable lack of tensile strength in jejunum ring segments as indicated by a lower level of passive tension in response to mechanical expansion
• more than 3-fold decrease in KCL- or carbachol-induced contractility in intestinal ring segments
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• failure of urine emptying into the bladder
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digestive/alimentary system
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• mice show a defect in dense bodies of the intestine, although to a lesser extent than in the bladder detrusor SMCs
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• decrease in intestinal length, but no evidence of microcolon is seen
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• 100% of mice show enlargement of the stomach after parturition and the first feeding
• however, few histological changes are seen in the intestine and stomach
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• more than 3-fold decrease in KCL- or carbachol-induced contractility in intestinal ring segments indicating intestinal hypoperistalsis
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• most likely caused by intestinal hypoperistalsis
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cellular
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• reduction in filamentous actin
• however, no ultrastructural actin filament perturbations are seen in the bladder or intestine
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| megacystis-microcolon-intestinal hypoperistalsis syndrome | DOID:0060610 |
OMIM:155310 |
J:241933 | |
