Analysis Tools
mortality/aging
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• lower than expected Mendelian ratio of homozygous mutants observed at E18.5 and day 0 postpartum indicating that partial lethality started at the perinatal stage
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• only one-fifth of the expected Mendelian ratio of homozygous mutants were alive at weaning
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renal/urinary system
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• numerous cysts of various sizes replacing most of the renal parenchyma in adults
• segment-specific markers indicated that the cystic changes primarily originated from glomeruli and proximal tubules during the maturation of induced nephrons
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• bilaterally in adults
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• extremely dilated calyces bilaterally in adults
• this changes were not due to mechanical obstruction
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pale kidney
(
J:132020
)
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• bilaterally in adults
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respiratory system
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• adult mice displayed reduced expression of an endothelial cell marker gene, suggeting impaired development of the distal lung vascular system
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• after fixation, mutant lungs were grossly larger than wild-type lungs
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• at 2 months of age, the numbers of macrophages and lymphocytes in the bronchoalveolar lavage fluid were significantly higher than those in control mice
• mice showed evidence of chronic inflammation
• small focal areas with inflammatory changes were observed in some lungs
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alveolitis
(
J:164957
)
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• focal areas of alveolar space filled with inflammatory cells (mostly macrophages) were observed at 9 months of age
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• most mutant lungs developed normally until birth; however, some appeared smaller and immature relative to wild-type lungs
• defects in distal lung morphogenesis are mainly observed during the saccular (E17 to P5) and alveolar (P5 to P14) stages
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• reduced alveolar compartmentalization is detected after P5 and clearly evident by 3 months of age, indicating impaired development of secondary alveolar septa
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• at 9 months of age, mutant alveolar walls appeared somewhat thin and fragile
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• enlarged air spaces were detected in mutant lungs at 8-9 months of age
• the mean linear intercept, used as a measure of interalveolar wall distance, was significantly greater in mutants
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• significantly enlarged air spaces and alveolar wall disruption, highly reminiscent of pulmonary emphysema, were noted at 8-9 months
(J:132020)
• focal areas of alveolar space filled with inflammatory cells (primarily macrophages) along with emphysema-like fibrotic changes and alveolar wall disruption were observed at 9 months of age
(J:164957)
• the mean linear intercept and destructive index were both significantly increased at 9 months of age
(J:164957)
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• after fixation with formalin, mutant lungs appeared somewhat paler than wild-type lungs
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• at 9 months of age, a white fibrotic change was observed at the base of some mutant lungs
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• a significant reduction in lung elastance was observed at 8 months
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• the deflation limbs of pressure-volume curves showed significantly increased lung volumes at all pressures examined
• the air volume injected at 26 cm H2O was significantly increased
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homeostasis/metabolism
immune system
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• at 2 months of age, the numbers of macrophages and lymphocytes in the bronchoalveolar lavage fluid were significantly higher than those in control mice
• mice showed evidence of chronic inflammation
• small focal areas with inflammatory changes were observed in some lungs
|
alveolitis
(
J:164957
)
|
• focal areas of alveolar space filled with inflammatory cells (mostly macrophages) were observed at 9 months of age
|
cardiovascular system
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• adult mice displayed reduced expression of an endothelial cell marker gene, suggeting impaired development of the distal lung vascular system
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growth/size/body
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• numerous cysts of various sizes replacing most of the renal parenchyma in adults
• segment-specific markers indicated that the cystic changes primarily originated from glomeruli and proximal tubules during the maturation of induced nephrons
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• bilaterally in adults
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• after fixation, mutant lungs were grossly larger than wild-type lungs
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