KVPY SA Exam 2014 Question Paper

Phenyl Ketonuria is an autosomal recessive disorder with mutation in gene for enzyme phenyl alanine hydroxylase (PAH), rendering it non functional
Phenyl alanine $\underset{\mathit{X}}{\overset{\mathrm{PAH}}{\mathit{–}\mathit{–}\mathit{–}\mathit{–}\mathit{–}\mathit{–}▸}}$ Tyrosine
→ Such person cannot metabolise the above reaction leading to accumulation of Phenyl alanine. So, are given food low in (Phe) and supplemented with (tyr).