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Jan 27, 2022 (publication date) through Oct 30, 2025
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World Journal of Hepatology
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1948-5182
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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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Copyright ©The Author(s) 2021.
World J Hepatol. Jan 27, 2022; 14(1): 180-194
Published online Jan 27, 2022. doi: 10.4254/wjh.v14.i1.180
Figure 1
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Figure 1 Carnitine shuttle. CoA: Co-enzyme A; FATP: Fatty acid transport protein; OCTN2: Organic cation transporter 2; CPT: carnitine palmitoyltransferase; CACT: Carnitine acyl carnitine transporter.
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Figure 2 Beta-oxidation of fatty acids. CoA: Co-enzyme A; NAD: Nicotinamide adenine dinucleotide; NADH2: Nicotinamide adenine dinucleotide (reduced); FAD: Flavin adenine dinucleotide; FADH: Flavin adenine dinucleotide (reduced).
Figure 3
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Figure 3 Urea cycle. NAGS: N-acetyl glutamate synthase; NAG: N-acetyl glutamate; CoA: Co-enzyme A; CPS-1: Carbamoyl phosphate synthetase 1; NH3: Ammonia; HCO3: Bicarbonate; ATP: Adenine triphosphate; OTC: Ornithine transcarbamoylase; ORNT: Ornithine transporter; ASS: Argininosuccinate synthetase; ASL: Argininosuccinate lyase.
Figure 4
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Figure 4 Approach to individual urea cycle defects. NAG: N-acetyl glutamate; CPS-1: Carbamoyl phosphate synthetase 1; OTC: Ornithine transcarbamoylase; ASS: Argininosuccinate synthetase; ASL: Argininosuccinate lyase; HHH: Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
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