Figure 6

Defective BA metabolism in taurine depletion by impairments in mt-tRNA modification and in BA conjugation. Taurine depletion decreases the taurine modified mt-tRNAs, and in turn, CYP27A1 protein located on the mitochondrial inner membrane is decreased. Bile acid syntheses, particularly in CDCA synthesis, are decreased according to the accumulated substrates and decreased metabolites of CYP27A1. Bile acid excretion into bile is decreased, and instead, the excretion into the peripheral circulation is increased.