It has been found that HSP90 preferentially accumulates in the mitochondria of PAH-PASMCs in response to stress, and the accumulation of mtHSP90 is a feature of PAH-PASMCs and an essential regulator of mitochondrial homeostasis, contributing to PAH vascular remodeling [88]. The gene discussed is HSP90AA1; the disease is pulmonary arterial hypertension.