Rexim's 17,000m2 Ham site mainly produces high quality pharmaceutical grades of amino acids, their salts and derivatives, as well as peptides and keto acids
Accumulation of these 3 amino acids and their corresponding keto acids
leads to encephalopathy and progressive neurodegeneration in untreated infants.
The amination of keto acids
is assumed to be more important then the elimination of ammonia (Fairbairn, 1957).
Poor preservation of samples will lead to nonenzymatic conversion of all keto acids
to the respective hydroxyacid; for example, acetoacetate is converted to 3-hydroxybutyrate, and 2-ketoglutarate is converted to 2-hydroxyglutarate.
During this reaction alpha keto acids
converted into amino acids may be utilized on the protein synthesis, thus regulating the carbohydrate and protein metabolisms (Knox and Greengard, 1965; Martin et al 1981).