Phenotypes Associated with This Genotype

Genotype
MGI:5430760

ht3

• Allelic Composition: Bmpr2^tm1Kmi/Bmpr2⁺
• Genetic Background: involves: 129S4/SvJae * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

cardiovascular system

abnormal pulmonary artery morphology ( J:98219 )

• heterozygotes exhibit an increase in wall thickness of muscularized pulmonary arteries

• heterozygotes exposed to hypoxia exhibit impaired muscularization of small pulmonary arteries in both the distal intra-acinar vessels and in proximal preacinar vessels, with 25% less increase in wall thickness compared to controls

abnormal lung vasculature morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes

• heterozygotes exposed to prolonged hypoxia exhibit impaired pulmonary vascular remodeling, with a smaller increase in pulmonary artery pressure, greater right ventricular hypertrophy, decrease in the number of alveolar-capillary units per millimeter squared, and decreased wall thickness (muscularization) of small pulmonary arteries compared to controls

increased pulmonary vascular resistance ( J:98219 )

• increase in mean total pulmonary vascular resistance and in incremental pulmonary resistance

• however total systemic vascular resistance is not different

increased pulmonary artery pressure ( J:98219 )

• increase in mean pulmonary arterial pressure

• however, mean systemic arterial pressure is not different from controls

• heterozygotes exposed to prolonged hypoxia show a smaller increase in pulmonary artery pressure than control mice

pulmonary hypertension ( J:98219 )

• mutants develop mild pulmonary hypertension

respiratory system

abnormal lung vasculature morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes

• heterozygotes exposed to prolonged hypoxia exhibit impaired pulmonary vascular remodeling, with a smaller increase in pulmonary artery pressure, greater right ventricular hypertrophy, decrease in the number of alveolar-capillary units per millimeter squared, and decreased wall thickness (muscularization) of small pulmonary arteries compared to controls

abnormal pulmonary alveolus morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes, decreasing the vessels-to-alveoli ratio