In Phenylketonuria the main aim of first-line therapy is
In PKU Phenylalanine hydroxylase enzyme is deficient.So alternative pathways are opened. Leading to the formation of Phenylketone,Phenyllactate and Phenylacetate. This can be prevented by limiting the substrate for deficient enzyme.Ref: DM Vasudevan - Textbook of Biochemistry, 7th edition, page no: 236
Replacement of the defective enzymes
Replacement of the deficient product
Limiting the substrate for deficient enzyme
Giving the missing amino acid by diet
Metabolism of protein and amino acid