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UK guidelines recommend monitoring clinical response and full blood count (typically at around 2 months) to ensure treatment adequacy, rather than routinely rechecking B12 levels after replacement has started

This model proposes that mGSH depletion due to its covalent binding to NAPQI acts as a first-hit, which is followed by a burst of mitochondrial ROS production that activates a second hit involving mitogen-activated protein kinases (MAPKs) that in turn amplifies the wave of mitochondrial ROS generation (Lemasters, 1998)

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In many cases, they do not reproduce the neurological phenotype of patients (Huszthy et al., 2012
