Figure 4. Carnitine metabolism and fatty acid oxidation are significantly increased in the PAH lung.

Multiple carnitine metabolites that flow into the fatty acid oxidation pathway are shown. Gene array shows that expression of genes coding for the key enzymes that catalyze particular steps in the pathway are significant increased, specifically Fatty Acetyl CoA synthase L1 (ACSL1); Acyl-CoA dehydrogenases isoforms M (ACADM; Acetyl –Coa Acetyl transferase 1 (ACAT1); Acetyl CoA Carboxylase isoform A (ACACA) short chain, medium chain, short/branched chain, and very long chain; and malonyl CoA decarboxylase. Metabolic intermediates for which significant differences were detected between NL and PAH are shown with a red frame (p<0.05); N = 8 for each box).