Pyruvate dehydrogenase kinase (PDK), a negative regulator of the mitochondrial enzyme pyruvate dehydrogenase (PDH), was upregulated in the wall of muscularized pulmonary arteries and remodeled RV from PAH patients compared to non-PAH donors, indicating the inhibition of glucose oxidation and upregulation of glycolysis in the pulmonary arteries and RV of PAH patients [109,110,111]. The gene discussed is PDP1; the disease is pulmonary arterial hypertension.