PDSS2 and nephrotic syndrome: While our results suggest that rapamycin administration is not a therapeutic option in primary CoQ deficiency, a recent study in the mouse model of CoQ deficiency due to a point mutation in the CoQ biosynthetic gene Pdss2 (the Pdss2kd/kd model) showed that oral administration of high doses of rapamycin (225 ppm) reduced the albuminuria in this mouse model of nephrotic syndrome [37].