OPA1 and pulmonary arterial hypertension: Ma et al. (2017) showed that hypoxia upregulated the expression of MFN1 in PASMCs both in vivo and in vitro, and that MFN1-mediated mitochondrial homeostasis and PASMCs proliferation were regulated by miR-125α. This also provides a theoretical basis for the treatment of PAH through miR-125α-MFN1 pathway. Recently, researchers found that O-[3-piperidino-2-hydroxy-1-propyl]-nicotinic amidoxim (BGP-15) promotes mitochondrial fusion by promoting the GTPase activity and self-aggregation of OPA1, but this role has not been confirmed in PAH (Szabo et al., 2018).