Genetic mutations that cause combined deficiencies in OATP1B1/OATP1B3 (Rotor Syndrome), result in redirection of CPI and CPIII elimination from bile to urine and an increase in CPI/CPIII urinary ratio (Wolkoff et al., 1976; van de Steeg et al., 2012). This evidence concerns the gene SLCO1B3 and Rotor syndrome.