In the NFU1 gene G206C mutation-induced PAH model, upregulation of the transporters CD36 and CPT1A promotes extracellular FA uptake and mitochondrial transport; Acyl-CoA synthetase long-chain family member 1 upregulation further enhances FAO, maintaining a hyperproliferative phenotype and ultimately driving PAH vascular remodeling [126]. This evidence concerns the gene NFU1 and pulmonary arterial hypertension.