cardiovascular system
• mutants develop marked pulmonary vascular remodeling as indicated by increased density of muscularized pulmonary vessels
• the increased density of muscularized pulmonary vessels becomes more prominent with time
• mutants show an increase in proliferation of smooth muscle cells in distal pulmonary vessel walls
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• mutants show an increase in proliferation of smooth muscle cells in distal pulmonary vessel walls
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• right ventricle to left ventricle and septum weight ratio is increased indicating right ventricle hypertrophy
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• right ventricular systolic pressure is increased
• however, systemic arterial pressure and heart rate are normal
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• right ventricular systolic pressure and right ventricle to left ventricle and septum weight ratio is increased indicating presence of sustained pulmonary hypertension
• pulmonary hypertension progression gets worse over time
• 20 week old mutants exposed to an acute hypoxic challenge (8% oxygen) do not exhibit the marked increase in right ventricular systolic pressure that is seen in wild-type mice, indicating depressed hypoxic pulmonary vasoconstriction
• 8 week old mutants exposed to chronic hypoxia (10% oxygen) exhibit a more severe increase of right ventricle systolic pressure and degree of right ventricle hypertrophy and distal pulmonary vessel muscularization than wild-type mice
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muscle
• mutants show an increase in proliferation of smooth muscle cells in distal pulmonary vessel walls
|
• right ventricle to left ventricle and septum weight ratio is increased indicating right ventricle hypertrophy
|
respiratory system
• mutants develop marked pulmonary vascular remodeling as indicated by increased density of muscularized pulmonary vessels
• the increased density of muscularized pulmonary vessels becomes more prominent with time
• mutants show an increase in proliferation of smooth muscle cells in distal pulmonary vessel walls
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growth/size/body
• right ventricle to left ventricle and septum weight ratio is increased indicating right ventricle hypertrophy
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
primary pulmonary hypertension | DOID:14557 |
OMIM:178600 OMIM:265400 OMIM:615342 OMIM:615343 OMIM:615344 |
J:144126 |