Here we show that PDH E1α subunit significantly increased in HD-iPSC, but serines 232, 293 and 300 residues were highly phosphorylated in both HD iPSC and NSC, indicating decreased activity of this enzymatic complex; indeed, PDK1 mRNA levels were upregulated and PDP1 downregulated in HD iPSC, consistent with enhanced PDHE1α phosphorylation, as identified in distinct HD models and patient’s brain tissue (Sorbi et al., 1983; Ferreira et al., 2011; Naia et al., 2017). Here, PDK1 is linked to Huntington disease.