Malate-fumarate transformation

In this way, it would be possible to convert electrodialytically fumaric acid into ammonium fumarate. This in turn may be enzymatically transformed into ammonium malate, which might finally be ED freed into malic acid with no reagent consumption and by-product formation, and minimum product loss. 

C-IO) Fumarase deficiency. There is a deficit in the transformation of fumarate to malate. The infant has developmental retardation, with abnormal neuromuscular function, lactic acidemia, and fumarate aciduria. The lactic acidosis may result from a backup of Krebs cycle function, all the way to lactate. Lactic acidosis may also be present in rare disorders of cytochrome oxidase activity. Diagnostically, there is a deficit in fumarase activity in assay of liver and skeletal muscle mitochondria. 

In the presence of oxygen and the cytochrome system, succinate is finally transformed to fumarate by a dehydrogenation process, and in turn, fumarate is converted into malate, which itself is dehydrogenated to form oxaloacetate. 

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