There are data indicating a relevant connection between improper intracellular CoA handling and myopathies: mutations in the PPCS gene, coding for the enzyme catalyzing the second step in CoA biosynthesis (Figure 1), cause an autosomal recessive dilated cardiomyopathy [53], whereas defects in SLC25A42, the mitochondrial transporters of CoA, leads to variable clinical phenotype with myopathy [54,55]. Here, PPCS is linked to myopathy.