Disorders such as PKU

Welcome to my Web site for  Low-protein cooking.

Low-protein food is recommended to persons with particular types of metabolic disorders such as  Phenylketonuria (PKU), Homocystinuria (HCU),  Methylmalonic Acidemia(MMA) and Tyrosinemia. Each of these disorders require food products which are low in particular types of amino-acids. i.e. phenylalanine in the case of PKU, methonine in the case of HCU. However it is not easy to find high protein foods with low content of these amino acids. Hence, the patients are given low-protein foods.

A simple explanation of the disorders such as  PKU  is given below:

 ____________________________________________________


           |

          V
     Gene AB                    Gene BC                   Gene CD



           |                                    |                                   |
          V                                  V                                 V


   Enzyme AB             Enzyme BC                 Enzyme CD


           |                                    |                                    |
           |                                    |                                    |
           |                                    |                                    |
          V                                  V                                  V
A----------------> B -------------------------> C --------------------------> D

                                                                                    |

                                                                                    |

                                                                                    |

                                                                                   V

                                                                                    --------------> E 




____________________________________________________

In a normal person, A is converted to D through intermediates B and C using enzymes AB, BC and CD as shown above. If enzyme AB is absent A will not be converted to B. This leads to accumulation of A which may be toxic and a deficiency in B which may be an essential nutrient.

For example, in Phenylketonuria (PKU),  phenylalanine(PHE)  is not converted to form tyrosine (TYR). This leads to accumulation of  PHE which is toxic and  deficiency of TYR which is an essential nutrient. In order to reduce the toxicity only low-protein diets which are low in  PHE (for PKU) are given regularly. The protein required to maintain proper growth,  is given by supplements which are free from  PHE.

While this website helps one in preparing low-protein dishes , I have not discussed the amount of protein a person can have each day. This information should be obtained from a physician or a dietician. However, each recipe gives the nutrient contents( based on Amino Acid Analyzer v 3.2,a software provided by Ross Laboratories).

Leave a Reply

Your email address will not be published. Required fields are marked *