Figure 1.

CoA homeostasis. Schematic description of the intracellular de novo CoA biosynthetic (A) and degradation pathways (B). Molecules shown to be able to correct defects due to a blockage in the first step of CoA biosynthesis (C). PANK, pantothenate kinase; Abbreviations: PPCS, phosphopantothenoylcysteine synthetase; PPCDC, phosphopantothenoylcysteine decarboxylase; PPAT, phosphopantetheine adenyltransferase; DPCK, dephospho-CoA kinase; ENPP, ectonucleotide pyrophosphatase/phosphodiesterase.