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Orotic aciduria (increased synthesis of orate) can occur due to a defect in the urea cycle and a defect in:

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Beta oxidation



A male infant who appeared normal at birth began to develop lethargy, hypothermia and apnea at 42 hours after delivery. The infant was found to have normal levels of blood glucose, blood pH = 7.45, and elevated levels of blood ammonium and glutamine.

You determine this infant has a disorder is due to a deficiency in ornithine transcarbamoylase. Based on this diagnosis, which lab values listed below would be consistent with this diagnosis?

Elevated levels ortic acid

Decrease n-acelyglutamte

Increase argine


Which of the following is first step in the movement of free ammonia from peripheral tissues (not skeletal muscle) to the liver AND is paired with the correct enzyme:

NH3 glutamate – glutamine glutamate syn

NH3 + glutamate – glutamin glutaminase

Pyruvate glutamate – alanine glutamate syn


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