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High aromatic amino acid concentration results from impaired hepatic clearance and increased production caused by muscular breakdown exacerbated by hyperglucagonemia.28, 31 The decreased concentration of branched-chain amino acids results from their increased utilization for gluconeogenesis.28, 32 This amino acid imbalance may result in the development of false neurotransmitters (e.g., octopamine) in the brain, which contribute to the development of HE.33, 34 These false neurotransmitters have a fraction of dopamine's excitatory effect on dopamine receptors

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Our aim was to develop a representative in vitro model of HT1 using human induced pluripotent stem cell (iPSC) and assess the effect of NTBC treatment