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The low activity of GDH3 is not attributed to its promoter but to dissimilarities in the amino acid sequence between the two expressed Gdh isoforms
(See figure below showing the methylation and transsulfuration pathways.) Glutathione production starts with the amino acid cysteine
Cardiomyopathy, cardiac arrhythmia, (likely due to the accumulation of long-chain fatty acids and acylcarnitines that cannot be oxidized), cardiac insufficiency and respiratory distress arise from CAC deficiency [120]
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