Analysis Tools
mortality/aging
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• survival rate is decreased after birth
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• less than 20% survival rate by P20
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growth/size/body
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• body weight of surviving mice increases more slowly from P1 onwards with mice showing a severe delay to thrive
• mice that reach post-weaning stages gain body weight continuously but only reach body weights of controls at mature stages
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digestive/alimentary system
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• an elongated shape of the transition zone from the antrum to the duodenum is seen
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• mispositioning of the intestine in mature (P40) abdomen
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• mice exhibit mis-positioning of the duodenum and cecum, indicating malrotation
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• mice exhibit mis-positioning of the cecum, indicating malrotation
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• complete absence of gap junctions in the circular smooth muscle layer of the intestine
• impaired and uncoordinated motility and cell-cell communication in intestinal smooth muscle cells
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• severe dilation of the cecum, which appears smaller
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• severe dilation of the small intestine
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• mice exhibit mis-positioning of the duodenum, indicating malrotation
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• slight, but significant, increase in intestinal length
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• severely delayed passage of meconium in the intestines, with meconium only reaching the ileum at E18.5 compared to being located in the colon in controls
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• mice show lack of coordinated contraction of the intestine and decreased motility of intestine
• intestinal tissue does not contract as narrow as control intestines
• intestines exhibit reduced amplitudes of pendular movements, with dueodenum having a 6.5-fold decrease and jejunum having a 17-fold decrease in the amplitude of movement
• however, frequency of contraction is normal
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• mice show delayed transport of milk from the stomach into and along the intestine in newborns, indicating impaired transport of chime
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• calcium transients in smooth muscle layers of intestine are uncorrelated
• however, neurotransmission is not impaired
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muscle
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• complete absence of gap junctions in the circular smooth muscle layer of the intestine
• impaired and uncoordinated motility and cell-cell communication in intestinal smooth muscle cells
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• mice show lack of coordinated contraction of the intestine and decreased motility of intestine
• intestinal tissue does not contract as narrow as control intestines
• intestines exhibit reduced amplitudes of pendular movements, with dueodenum having a 6.5-fold decrease and jejunum having a 17-fold decrease in the amplitude of movement
• however, frequency of contraction is normal
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• cultured embryonic ureters are unable to form proximal-to-distal directed peristaltic waves and in extended culture, predominantly uncoordinated, fibrillation-like movements of the ureter wall without full contractions are seen, indicating impaired ureteral motility
• impaired and uncoordinated motility and cell-cell communication in ureteral smooth muscle cells
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• smooth muscle cells in the gastrointestinal tract are able to contract and show increased tone, with individual thin ring preparations of pyloric sphincter showing increased maximal contraction force and fundus strips and rings of lower esophageal sphincter showing higher muscle tone and reduced sensitivity towards nitric oxide
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renal/urinary system
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• urinary level of NGAL (neutrophil gelatinase-associated lipocalin; also termed lipocalin2), a marker of renal tubular injury, is already increased at early postnatal stages
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• bilateral hydronephrosis develops perinatally and aggravates rapidly by dilation of the renal pelvis and loss of renal parenchyma
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• marker analysis indicates renal tubular injury and the concentration of urinary neutrophil gelatinase-associated lipocalin which is rapidly and massively induced during renal injury is increased
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• impaired and uncoordinated motility and cell-cell communication in ureteral smooth muscle cells
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• calcium transients in smooth muscle layers of the ureter are uncorrelated
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• cultured embryonic ureters are unable to form proximal-to-distal directed peristaltic waves and in extended culture, predominantly uncoordinated, fibrillation-like movements of the ureter wall without full contractions are seen, indicating impaired ureteral motility
• impaired and uncoordinated motility and cell-cell communication in ureteral smooth muscle cells
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homeostasis/metabolism
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• urinary level of NGAL (neutrophil gelatinase-associated lipocalin; also termed lipocalin2), a marker of renal tubular injury, is already increased at early postnatal stages
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