mortality/aging
• mice die by P1
|
digestive/alimentary system
• at E14.5, mice exhibit a progressive defects in elongation compared with wild-type mice
• at E14.5, distal intestine contains a dilated gut lumen unlike in wild-type mice
|
• posterior gut region abnormalities are evident as early as E12.5
|
• terminal differentiation is severely impaired
|
• the intestine ends in an abnormal distal structure that terminates in a blind-sac
• mice exhibit intestinal obstruction unlike in wild-type mice
• at E16.5, mice exhibit a severe shortening of the intestine unlike in wild-type mice
• the posterior intestine is anteriorized
|
• terminal differentiation is severely impaired
|
• proximal and medial intestinal epithelia is less organized compared to in wild-type mice
• the cuboidal epithelia of the jejunum and ileum is replaced with a flattened epithelium
• duodenum epithelial cell proliferation is increased more than 20% compared to in wild-type mice
• however, apoptosis rates of duodenum epithelial cells are normal
• differentiation of gastric glandular epithelial cells cannot be detected
• posterior intestinal epithelial cells contain tonofilaments unlike in wild-type cells
|
• terminal differentiation is severely impaired
|
• the mutant ileum and cecum lack villi entirely
|
absent colon
(
J:149479
)
• no colon forms
|
• the duodenum contains villus-like epithelial foldings that are stunted and broadened compared to in wild-type mice
• duodenum epithelial cell proliferation is increased more than 20% compared to in wild-type mice
• however, apoptosis rates of duodenum epithelial cells are normal
|
• the duodenum becomes progressively distended and translucent likely due to fluid retention caused by distal obstruction
• by E18.5, the duodenum is dilated 5- to 7-fold compared to in wild-type mice
|
• at E16.5, mice exhibit villus hypoplasia
• at E18.5, reduced villus increases in severity from anterior to posterior
• the mutant ileum and cecum lack villi entirely
|
cellular
• terminal differentiation is severely impaired
|