RESUMO
The aim of the present study was to synthesize two low-phenylalanine diet formulae. These formulae were to be readily available from natural milk [skim milk] using two proteolytic purified enzymes [immobilized papain and protease XXIII]. Skim milk hydrolysate was adsorbed on either barium sulphate or on activated carbon. The formula was supplying 0.71 g phenylalanine/100 g protein and 0% free phenylalanine in the formula adsorbed on barium sulphate compared with 3.41% in the formula adsorbed on activated carbon. The effects of such two formulae on the cellular pathology, lipid and protein profiles were studied in an animal PKU model. BALB/c mice were used to induce hyperphenylalaninemia using 3% L-phenylalanine and 0.3% L-ethionine. The study concluded that skim milk hydrolysate formulae proved to be effective in reducing the blood level of phenylalanine and restoration of the cellular normal pattern as well as cellular lipids and protein profiles. These formulae may be helpful in the implementation of dietary management of PKU children and the hyperphenylalaninemia mothers, especially before conception and during pregnancy