Specifically in PS patients, CoQ10 deficiency could have an association with the aforementioned common V343A variant in the COQ2 gene in sporadic MSA [73], mutation in the NUS1 gene [74] (influencing the dolichol biosynthetic pathway that shares common biosynthetic steps with Q10), a hypothesized dysregulation of the specialized lipid metabolism involved in myelin synthesis and maintenance in MSA [75], or unknown individual genetic backgrounds (e.g., different mtDNA haplogroups) [76]. This evidence concerns the gene NUS1 and hyperinsulinemic hypoglycemia, familial, 4.