The results in Table 2 suggest that the elevation in serum cholesterol that is characteristic of Pdss2kd/kd homozygotes [20] is not entirely attributable to kidney disease, but likely results from shunting of farnesyl diphosphate (one of the precursors in the CoQ biosynthetic pathway immediately proximal to the Pdss2 enzymatic block) to alternative pathways, primarily involving cholesterol biosynthesis. Here, PDSS2 is linked to kidney disorder.