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Following conjugation, GSH-conjugated metabolites are exported via multidrug resistance-associated proteins (MRPs) and processed through the mercapturic acid pathway for renal excretion

Organic Acid Testing includes indirect markers such as pyroglutamic acid that may reflect increased oxidative stress or reduced glutathione recycling capacity

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Parenteral Vitamin B12 is the recommended treatment and will be required for the remainder of the patient's life