Analysis Tools
mortality/aging
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• death between 11 and 20 hours after birth
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behavior/neurological
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• lack of milk in neonatal stomach
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• failure to suckle
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cardiovascular system
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• extensive intrapulmonary and pleural hemorrhage, suggesting fragile pulmonary capillaries or veins
(J:29902)
• abnormally dilated intrapulmonary blood vessels seen at E18.5
(J:111447)
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• abnormal dilation of intrapulmonary veins noted as early as E14.5
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hemothorax
(
J:29902
)
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• extensive pleural hemorrhage
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• extensive intrapulmonary hemorrhage
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craniofacial
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• mutant palatal shelves elevate normally and meet at the midline, but there is no evidence of medial edge epithelial fusion
• the medial edge of the clefted palatal shelves is surfaced with a stratified squamous epithelium
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• in half of the homozygotes, the cleft proceeds into the most anterior part of the palate
• in other mutants, the anterior segment is fused
• the primary palate is not fused to the secondary palate in many cases
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homeostasis/metabolism
respiratory system
|
• extensive intrapulmonary and pleural hemorrhage, suggesting fragile pulmonary capillaries or veins
(J:29902)
• abnormally dilated intrapulmonary blood vessels seen at E18.5
(J:111447)
|
hemothorax
(
J:29902
)
|
• extensive pleural hemorrhage
|
|
• extensive intrapulmonary hemorrhage
|
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• delayed embryonic, fetal and neonatal development of the airways and terminal airspaces, first detected at E12.5
(J:29902)
• 50% reduction in number of epithelial tubules seen at E12.5
(J:29902)
• 50% reduction in number of primitive peripheral branches seen at E14.5; dilated peripheral branches with increased mesenchymal thickness
(J:29902)
• immature lung phenotype, including pseudoglandular histology with alveolar hypoplasia, mesenchymal thickening and hypercellularity seen at birth
(J:29902)
• delayed saccule formation with increased mesenchymal thickness between the terminal air spaces seen at E18.5
(J:111447)
• normal size and lobation of fetal lungs seen at E18.5
(J:111447)
• dexamethasone treatment on E15.5 and E16.5 promoted saccular structure formation; however, morphological maturation of mutant fetal lungs appeared to be retarded relative to that of similarly treated wild-type fetal lungs
(J:111447)
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• alveolar hypoplasia and lack of alveolar septal formation in neonatal lungs
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• decreased number of saccular structures seen at E18.5
• atypical saccular structures with poorly dilated tubules, thickened surrounding mesenchyme, and fewer projections (buds for septal formation) extending into the lumen
• saccular structures are restricted to a narrow region directly below the pleural membrane
• dexamethasone treatment enhanced saccular formation in both mutant and wild-type fetal lungs; however, the effect was greater in wild-type fetal lungs
|
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• dilated peripheral branches with increased mesenchymal thickness at E14.5
(J:29902)
• mesenchymal thickening and hypercellularity seen at birth
(J:29902)
• increased mesenchymal thickness between the terminal air spaces at E18.5
(J:111447)
|
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• alveolar hypoplasia
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• underdeveloped and poorly inflated alveoli in neonatal lungs
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• decreased number of type II alveolar epithelial cells in neonatal lungs, as revealed by immunostaining for surfactant protein proSP-C
(J:29902)
• 30% reduction in relative number of SP-C-positive epithelial cells detected by immunostaining for surfactant protein proSP-C
(J:111447)
• 50% reduction in SP-C mRNA expression levels seen at E18.5
(J:111447)
• dexamethasone treatment reduced the relative number of SP-C-positive epithelial cells in fetal lungs of both wild-type and mutant mice by 60%
(J:111447)
• dexamethasone treatment reduced SP-C expression by ~75% in wild-type lungs and 60% in mutant fetal lungs relative to that in control lungs
(J:111447)
|
atelectasis
(
J:29902
)
|
• observed in neonatal lungs
|
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• lack of alveolar septal formation
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• reduced amount of elastin fibers seen in fetal lungs at E18.5
• thinner elastin fibers seen only in a few saccular structures but not in most of tubule structures
• dexamethasone treatment increased intensity and number of stained elastin fibers in both wild-type and mutant fetal lungs
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• marked dilatation of the conducting airways in neonatal lungs
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• gasping for air prior to death
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digestive/alimentary system
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• mutant palatal shelves elevate normally and meet at the midline, but there is no evidence of medial edge epithelial fusion
• the medial edge of the clefted palatal shelves is surfaced with a stratified squamous epithelium
|
|
• in half of the homozygotes, the cleft proceeds into the most anterior part of the palate
• in other mutants, the anterior segment is fused
• the primary palate is not fused to the secondary palate in many cases
|
growth/size/body
|
• mutant palatal shelves elevate normally and meet at the midline, but there is no evidence of medial edge epithelial fusion
• the medial edge of the clefted palatal shelves is surfaced with a stratified squamous epithelium
|
|
• in half of the homozygotes, the cleft proceeds into the most anterior part of the palate
• in other mutants, the anterior segment is fused
• the primary palate is not fused to the secondary palate in many cases
|
