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Once diagnosed, replacing B12 can fully resolve PA symptoms

However, the labelling (M + 4) in metabolites produced after direct metabolism of [U- 13 C] glutamate such as fumarate, malate and aspartate was significantly decreased in FTD3 neurons compared to isogenic controls (with the exception of fumarate and -kg M + 4 in H150, Fig 3 c 25 ), in contrast to the first turn TCA cycle in which metabolites were unmodified in FTD3 neurons compared with isogenic controls

Yet, in studying GSH levels, Raffa et al

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