Amino acids are one of the most necessary substances in intracellular metabolic processes.aromatic aminoacids such as phenylalanine and tyrosine are precursor of melanin so are important materials for the skin pigmentation.metabolic disorders in the melanin synthesis causes milky macules on the skin of patients,known as vitiligo.these patients are hypersensitive to sunlight and consequently at ...

Methods routinely utilized for detection of phenylalanine in new-born blood consist of enzymatic assays, lacking sensitivity and HPLC assays which are expensive and time-consuming to conduct. We, here, report for the first time, the construction of a phenylalanine sensitive electrode, on the basis of a selective molecularly imprinted polymer, offering sensitivity, economy and ease of use for th...

The optimal dietary management of children with phenylketonuria (PKU) has rarely been rigorously explored. The aim of this study was to assess longitudinally the effects of three factors thought to influence plasma phenylalanine concentrations in PKU: total energy intake; protein intake from natural foods allowed freely in addition to allocated phenylalanine exchanges; and the distribution of p...

background: phenylalanine dehydrogenase (phedh ec 1.4.1.20) is a nad+-dependent enzyme that performs the reversible oxidative deamination of l-phenylalanine to phenylpyruvate. it plays an important role in detection and screening of phenylketonuria (pku) diseases and production of chiral intermediates as well. the main goal of this study was to find a simple and rapid alternative method for pur...

Phenylketonuria (PKU) is caused by deficiency of phenylalanine hydroxylase, resulting in an accumulation of phenylalanine in brain tissue and cerebrospinal fluid of phenylketonuria patients. Phenylketonuria is neuropathologically characterized by neuronal cell loss, white matter abnormalities, dendritic simplification, and synaptic density reduction. The neuropathological effect may be due to t...

The laboratory mouse represents an important model for the study of phenylalanine metabolism and the pathochemistry of phenylketonuria, yet mouse phenylalanine hydroxylase (PAH) has not been extensively studied. We report the cloning and sequencing of a mouse PAH cDNA, the expression of enzymic activity from the mouse PAH cDNA clone and the identification of mouse PAH and human PAH by two-dimen...