respiratory system phenotype

• abnormal pulmonary alveolar duct morphology
  • alveolar ducts comprise a significantly greater proportion of mutant lung tissue at P28, P42, and P56
  (MGI Ref ID J:110539)

• abnormal respiratory function
  • Normal - however, the pressure/volume characteristics of the lung are not significantly altered at this time point
  (MGI Ref ID J:110539)
  • carbon monoxide (CO) uptake is significantly reduced in mutant lungs at P56 when mice are at rest
  (MGI Ref ID J:110539)

• decreased pulmonary endothelial cell surface
  • the gas-exchange surface area per cm3 of mutant lung tissue is reduced by ~30% at P28, P42 and P56
  (MGI Ref ID J:110539)

• abnormal pulmonary alveolar sac morphology
  • alveolar sacs comprise a significantly greater proportion of mutant lung tissue at P28, P42, and P56
  (MGI Ref ID J:110539)

• abnormal pulmonary alveolar system morphology
  • Normal - no significant difference in the thickness of alveolar walls is observed at any time point
  (MGI Ref ID J:110539)
  • Normal - no differences in the percentage of BrdU-labeled cells are noted in the alveolar region at any of time point
  (MGI Ref ID J:110539)
  • Normal - however, the mean volume of individual alveoli, number of alveoli per cm3 of lung tissue, and distribution of alveolar volumes remain relatively normal at all postnatal time points studied
  (MGI Ref ID J:110539)
  • the volume density of airspace in the alveolar region of mutant lungs is significantly increased at P28, P42, and P56
  (MGI Ref ID J:110539)
  • the mean cord length (Lm) of mutant respiratory airspaces is significantly increased only at P42
  (MGI Ref ID J:110539)

• abnormal lung development
  • impaired formation of the distal airspaces during the postnatal phase of lung maturation
  (MGI Ref ID J:110539)

• impaired lung alveolus development
  • Normal - however, no differences in Pdgfra mRNA levels are observed at any postnatal age
  (MGI Ref ID J:110539)
  • significantly reduced PDGFA protein levels are observed at P28, suggesting impaired postnatal lung alveolarization
  (MGI Ref ID J:110539)

behavior/neurological phenotype

• abnormal spatial learning
  • mutants display severe impairment in the Morris water maze test and show no improvement in performance over a training period of 8 days; after 24 hours mutants spend less time in the water where the platform had been in the previous trials compared to wild type controls
  (MGI Ref ID J:51892)
  • d type controls
  (MGI Ref ID J:51892)

cardiovascular system phenotype

• decreased pulmonary endothelial cell surface
  • the gas-exchange surface area per cm3 of mutant lung tissue is reduced by ~30% at P28, P42 and P56
  (MGI Ref ID J:110539)

growth/size/body region phenotype

• decreased body weight
  • Normal - however, no significant differences in body weight are observed at P42 and P56
  (MGI Ref ID J:110539)
  • slightly but significantly decreased body weights are noted at P28
  (MGI Ref ID J:110539)

nervous system phenotype

• abnormal excitatory postsynaptic potential
  • only transient depression of field excitatory postsynaptic potentials is observed in mutants in response to low frequency stimulation, while the controls show a long-lasting depression
  (MGI Ref ID J:51892)

• reduced long term potentiation
  • long term potentiation is absent in hippocampal slices from mutants
  (MGI Ref ID J:51892)

• absent long term depression
  • long term depotentiation can not be induced in mutants
  (MGI Ref ID J:51892)