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These polysaccharides influence lipid metabolism through various pathways, as illustrated in Figure 5

In agreement, human PKU-derived lymphocytes showed a high Phe/Tyr ratio with insufficient mitophagy [125], revealing an intimate relationship between BH4 deficiency and impaired autophagy

Metabolism 45:15141520 Yang T, Adamson TE, Resnick JL, Leff S, Wevrick R, Francke U, Jenkins NA, Copeland NG, Brannan CI (1998) A mouse model for Prader-Willi syndrome imprinting-centre mutations