Most recently, it was demonstrated that significantly increased expression of Hsp110 occurred in the pulmonary arteries of mice and HPASMCs under hypoxia, and knockdown of Hsp110 would alleviate hypoxia-induced PAH in mice [24], which unraveled the mysterious role of Hsp110 in inducing vascular remodeling. Here, HSPH1 is linked to pulmonary arterial hypertension.