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Pulmonary Arterial Hypertension
Loss of function

Pulmonary Arterial Hypertension

  • Genotype

    BMPR2

  • Mechanism

    Loss of function

  • Phenotype

    Tolerance, Fatigue, Locomotor activity, Hypoxia test, Anxiety, Social isolation, Muscle strength

  • Biomarker

    Endothelin-1, VEGF-A, MMP 9, Fibrinogen

  • Gene Expression Markers

    id1, smad1, smad6, id1, enos, sm22α, myh11, hif1α

  • Physiology Changes

    Respiratory rate, Mean Pulmonary Arterial Pressure, ECG - Right Ventricular Hypertrophy

  • Histology Grading Endpoint

    Severe medial thickening, Extensive muscularization, Marked lumen occlusion, Plexiform-like endothelial lesions, Inflammation and Fibrosis

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