In primary pulmonary hypeension basic abnormality in genes lies in
Ref Harrison 19 th ed pg 1655 Abnormalities in multiple molecular pathways and genes that regu- late the pulmonary vascular endothelial and smooth muscle cells have been identified ). These abnormalities include decreased expression of the voltage-regulated potassium channel, mutations in the bone morphogenetic protein receptor-2, increased tissue factor expres- sion, overactivation of the serotonin transpoer, hypoxia-induced activation of hypoxia-inducible factor-1a, and activation of nuclear factor of activated T cells.
Bone morphogenetic protein receptor II
Endothelin
Homebox gene
PAX-11
C.V.S