Mouse Genome Informatics
tg
    Tg(Tagln-SLC6A4)#Edd/?
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
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
• 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
• right ventricular systolic pressure is increased
• however, systemic arterial pressure and heart rate are normal
• 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

muscle
• mutants show an increase in proliferation of smooth muscle cells in distal pulmonary vessel walls

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

Mouse Models of Human Disease
OMIM IDRef(s)
Pulmonary Hypertension, Primary, 1; PPH1 178600 J:144126