This compound increased mitochondrial respiration, causing upregulation of the NAD+/NADH and α-KG/2-hydroxyglutarate (2-HG) ratio, which in turn resulted in promoter CpG island demethylation, activating the differentiation program in MYCN-amplified NB.135 In addition, we previously showed that inhibition of fatty acid synthase (FASN) or acetyl-CoA carboxylase A (ACACA) with TOFA, Cerulenin, Orlistat (approved for treatment of obesity), Soraphen A, or UB006 (Figure 3), induced NB differentiation independently of MYCN status.101. Here, MYCN is linked to obesity due to melanocortin 4 receptor deficiency.