Document Type: Original Article
Department of Food Sciences & Technology, Faculty of Advanced Sciences & Technology, Pharmaceutical Sciences Branch, Islamic Azad University, Tehran, Iran.
Department of Food Sciences &amp; Technology, Faculty of Advanced Sciences &amp; Technology, Pharmaceutical Sciences Branch, Islamic Azad University, Tehran, Iran.
Phenylketonuria (PKU) is one of the most prevalent types of hereditary metabolic disorders which is caused due to an absence or reduction of the activity of the Phenylalanine hydroxylase enzyme in the liver which in turn, inhibits the transformation of phenylalanine (Phe) to tyrosine. In clinical terms, this disorder is displayed with severe, permanent and irreversible mental retardation. This research was aimed at development of a highly nutrient and acceptable suitable analogue Kefir drink for these patients. The mentioned drink is based on milk permeate, cream powder and includes glycomacropeptide (GMP) as a source of protein, starter as a fermentation source, the trance glutamines (TG) enzyme, dough stabilizer and modified corn starch as tissue maker, salt and water. The GMP used in this analogue drink is intended for enrichment of the product and therefore it was added by 3% to one formula. The aforementioned sample had a lower calculated amount of pH and alcohol percentage in comparison with the 16 samples which did not have GMP. The results of this study showed that the analog kefir has a low level of phenylalanine (30.40 mg/100g) and in that regard, it can be considered to be useful for patients with PKU.