Development of low phenylalanine flour for phenylketonuric patient

Abstract

Phenylketonuria is an autosomal recessive genetic disorder due to the mutation in the phenylalanine hydroxylase (PAH) gene which results in the lower level of phenylalanine hydroxylase enzyme, hence the conversion of phenylalanine (Phe) to tyrosine does not take place and excess Phe takes an alternate pathway to produce phenylketones. This manuscript describes the attempts made toward the development of a gelatin-based multipurpose flour with other low-cost ingredients as the available food products for PKU patients in the market are very expensive and affordability becomes an issue for the patients. The flour is easily bakeable, friable, extrudable, and can be mixed with an adequate amount of water to make new products. The Phe content of the sample was 251.8 mg/100 g of flour which was well within the limits, as recommended by the WHO. The experimentally developed biscuits from the flour has low Phe content and is suitable for PKU patients. Some of the applications for proposed low phenylalanine flour for phenylketonuric patient could be as a diet supplement for the patient suffering from PKU. The key advantage of this multipurpose flour is the low cost of the flour having high nutritional value. The flour can be used for baking, extrusion, and frying purpose. The functional and sensory properties of the biscuit formulated from the flour, are in par with commercially available biscuits indicating its suitability for commercialization. Furthermore, work on improving the flour quality and other functional properties of the flour can be studied. Practical applications The present article focuses on the importance of diet supplement for the patient suffering from PKU. The important advantage of multipurpose flour is the low cost of the ingredients and high nutritional value. The functional and sensory properties were in par with commercially available biscuit indicating its suitability for consumption. Further work on improving the flour quality and other functional properties of the flour can also be studied.