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In conclusion, an ATP-depleting futile 5-oxoproline cycle that can lead to the accumulation of 5-oxoproline is described and shown in Figure 5

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Carnitine deficiency can theoretically cause hyperammonemia, but in practice this seems to be a less important mechanism (especially among patients being treated with IV carnitine)

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Flare-ups often occur with sun exposure or hormonal changes, and each episode may last three months or longer

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