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Most patients experience symptomatic improvement within 1-2 weeks, with objective measures improving over 4-8 weeks

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This study found that in animals treated with 2-Aminobicyclo [2, 2, 1] heptane-2-carboxylic acid (BCH), a nonmetabolic analogue of leucine, the renal arteriovenous ammonia level change was greater than in the liver, indicating that hyperammonemia in GDH-HI patients is mainly induced due to increased renal ammonia production, instead of abnormalities in urea synthesis in the liver

After 6 h transfection, cells were changed with fresh medium containing 5% FBS

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