Recently, several reports suggested the involvement of Hsp90 in the pathogenesis of PAH, especially in vascular remodeling, although the precise pathogenesis mechanisms of PAH remain to be elucidated (Sakao and Tatsumi, 2011; Wang et al., 2016; Boucherat et al., 2017, 2018). Here, HSP90AA1 is linked to pulmonary arterial hypertension.