Via analyzing the TRPC6 gene promoter of pulmonary artery smooth muscle cells from patients with idiopathic pulmonary arterial hypertension (IPAH), three single-nucleotide polymorphisms are identified and one of them are found to increase basal gene promoter activity, which may link abnormal transcription of TRPC6 to the activation of NF-κB and lead to upregulated risk of IPAH16. Here, TRPC6 is linked to idiopathic pulmonary arterial hypertension.