Analysis Tools
mortality/aging
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• homozygotes die within 3-10 minutes of birth due to respiratory failure
• a few homozygotes survive up to ~4 hrs after birth
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respiratory system
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• newborn homozygotes show abnormal lung morphology with dense cellularity and increased cell proliferation in the distal airways
(J:82687)
• lung mesenchyme appears thicker than normal at E18.5
(J:119680)
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• newborn homozygotes display narrower and tortuous airspaces and decreased numbers of flattened epithelial cells relative to wild-type mice
(J:82687)
• alveolar sacs are very narrow and irregular at E18.5, but not at earlier times
(J:119680)
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• newborn homozygotes display significantly fewer attenuated alveolar epithelial type I cells, although structurally normal type II cells and secreted surfactant proteins are present
• type I cell differentiation appears to be blocked, as shown by reduced levels of aquaporin-5 mRNA and protein, a type I cell water channel
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atelectasis
(
J:82687
)
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• the lungs of homozygotes that die within a few min after birth are not inflated
• longer survivors exhibit partially inflated lungs with small volumes of air
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• newborn homozygotes display thicker interalveolar septae relative to wild-type mice
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• homozygotes exhibit respiratory failure; they gasp for breath but fail to inflate their lungs to normal volumes
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homeostasis/metabolism
lymphedema
(
J:94978
)
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• homozygotes exhibit congenital cutaneous lymphedema, as shown by thickened wrinkles, particularly in the neck area, and swelling of the lower extremities
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immune system
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• intradermal dye injection into mutant foot pads indicates only dilated subcutaneous lymphatic collectors but fails to visualize normal dermal capillary networks
• no dye is detected in mutant retroperitoneal para-aortic lymph nodes and lymphatic ducts, indicating impaired centripetal lymphatic transport
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• newborn homozygotes display impaired patterning of lymphatic capillary networks, as shown by an increased number of non-anastomozing, blind beginning cutaneous lymphatic capillaries in the ear skin
• notably, blood vessel pattern formation and epidermal differentiation/structure remain unaffected
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• newborn homozygotes exhibit severe dilation of both dermal and submucosal intestinal lymphatics, most likely due to the loss of connecting lymphatics between the superficial and deep capillary networks
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growth/size/body
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• newborn homozygotes are slightly heavier (19%) than wild-type mice; however, lung to body weight ratios remain normal
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behavior/neurological
cardiovascular system
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• intradermal dye injection into mutant foot pads indicates only dilated subcutaneous lymphatic collectors but fails to visualize normal dermal capillary networks
• no dye is detected in mutant retroperitoneal para-aortic lymph nodes and lymphatic ducts, indicating impaired centripetal lymphatic transport
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integument
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• newborn homozygotes display smoothened skin texture with thickened wrinkles in the neck area
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