Gene expression profiles of Leigh syndrome mouse samples and Ndufs4 knockout human iPSCs before and after Pioglitazone treatment

Leigh syndrome is a progressive neurodegenerative disease caused by knockout (KO) of the Ndufs4 gene, leading to mitochondrial oxidative stress, inflammation and aberrant mitochondrial dynamics. Our previously study found that pioglitazone could suppresses neuroinflammation and oxidative stress both in vivo and in vitro. In this study, we investigate the gene expression of medulla, cortex and hippocampus from Ndufs4 KO mouse that had been treated with pioglitazone. Moreover, we analysis the RNA expression on pioglitazone treated Ndufs4 deficient human iPSCs. Overall design: In total, 34 mouse and 11 human bulk RNA-seq samples were profiled. In the mouse, 18 samples were from the medulla (including 10 from knockout and 10 from WT), 8 from hippocampus (including 4 from knockout and 4 from WT), and 8 from cortex (including 4 from knockout and 4 from WT). In each of these 6 mouse groups (tissue and genotype), half of the samples were treated with pioglitazone and the others without. In the human, the samples were based on neural progenitor cells (NPCs) derived from hESC-ES1 with or without NDUFS4 single or double knockout.