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Co-labelling for the motor neuron marker choline acetyl-transferase and for SMN revealed that at 1 month of age, an age prior to development of pathology or abnormal motor phenotypes, expression of mutant R521C had already provoked a slight reduction of Gems (Fig

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The rate of GSH synthesis is dependent upon the availability of cysteine and the activity of the rate-limiting enzyme, -glutamylcysteine synthetase (also called glutamate-cysteine ligase, GCL)

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For patients exhibiting non-responsiveness to Liposomal Glutathione