Author/Authors :
Mohamed, Hoida A. Agriculture Research Center - Food Technology Institute, Egypt , Osman, Hoda M. Ain Shams Univ. - Fac of Medicine - Clinical Chemical Pathology Dept, Egypt , EI-Magdoub, Mohamed N. I. Ain Shams Univ. - Fac Agric - Food Sconce Dept , Shehata, Abdou E. Ain Shams Univ. - Fac Agric - Food Sconce Dept, Egypt
Abstract :
Phenylketonuria is the most common genetic inborn error of amino acid metabolism. The accepted treatment is to restrict dietary intake of phenylalanine. Protein substitute is an essential component in the management of PKU. The aim of the present study was to synthesize two low-phenylalaninediet 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 gm phenylalanine 1100 gram protein and 0% free phenylalanine in the formula adsorbed on barium sulphate compared to 3.41 % in the formula adsorbed on activated carbon. The effects of such two formulae on the celIular pathology, lipid and protein profiles have been studied in an animal PKU model. BALB/c mice were used to induce hyperphenylalaninemia using 3% L-phenylalanine and 0.3% Lethionine.
