Extended Data Fig. 8: Disease mutant A42N291D and mitochondrial matrix-targeting MTS-COASY cannot rescue loss of mito CoA transporter in leu5 deletion yeast strain.

a, Anti-MYC and anti-FLAG western blot of mitochondria lysates from leu5△ yeast expressing FLAG-tagged Leu5, MYC-tagged hCOASY and mitochondrial targeting MTS-hCOASY. Safe stain, loading control. Blots are representative data from experiments that were independently repeated for at least two times with similar results. b, Yeast complementation assay of 10-fold serial dilutions of leu5Δ cells expressing Leu5, hCOASY, MTS-hCOASY, Leu5+hCOASY, or Leu5+MTS-hCOASY in glucose (fermentable) or glycerol (unfermentable) fuel source. c, Yeast complementation assay of 10-fold serial dilutions of leu5Δ cells expressing Leu5, hSLC25A42WT (A42WT), hSLC25A42N291D (A42N291D), hSLC25A16WT (A16WT) in glucose (fermentable) or glycerol (unfermentable) fuel source. As positive control in yeast complementation assay (b and c), wild type strain BY4741 transformed with empty vector was used.