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Due to abnormal intracranial PLP transport in patients with TNSALP mutations, there is insufficient neurotransmitter synthesis in the brain, which eventually contributes to PL-dependent seizures, usually preceding the onset of skeletal changes [41, 49,50,51,52]

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Furthermore, in bile ductcannulated (BDC) rats, the average recovery rates of total radioactivity in bile, urine, feces, and cage cleaning fluid collected during 72 h after dosing were 9.08% 0.86%, 17.77% 6.35%, 2.73% 0.40%, and 0.91% 0.13%, respectively (Table 8

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