Phenylketonuria (PKU) is an inborn error in the metabolism of the amino acid phenylalanine (Phe) due to the deficiency of an enzyme phenylalanine hydroxylase (PAH). Current therapy consists of a Phe – restricted diet for life to ensure the healthiest development. It is particularly important for PKU women in the reproductive age group to comply with the diet, since elevated maternal blood Phe l...

To determine the relationships among the processes of uptake, intracellular pool formation, and incorporation of amino acids into protein, we measured the uptake of dipeptides and free amino acids by bacterial assemblages in estuarine and coastal waters of the southeast U.S. The dipeptide phenylalanyl-phenylalanine (phe-phe) lowerd V,,,,, of phenylalanine uptake when the turnover rate of phenyl...

Phenylketonuria (PKU) is an autosomal recessive disorder of phenylalanine (Phe) metabolism, causing a build-up Phe in the body. Treatment consists Phe-restricted diet for life and regular determination blood levels to monitor intake Phe. Despite fact that cornerstone treatment, there are no studies examining common knowledge about food items whether they allowed as part PKU diet. Improving pare...

Phenylketonuria (PKU) is an autosomal recessive genetic disorder in which mutations in the phenylalanine-4-hydroxylase (PAH) gene result in an inactive enzyme (PAH, EC 1.14.16.1). The effect is an inability to metabolize phenylalanine (Phe), translating into elevated levels of Phe in the bloodstream (hyperphenylalaninemia). If therapy is not implemented at birth, mental retardation can occur. P...

L-phenylalanine (Phe), an essential amino acid, is necessary for the synthesis of proteins such as catecholamine and melanin, also precursor acid L-tyrosine (Tyr). In this paper, optimized structures Phe, were calculated by using semi-empirical models AM1, PM3 MNDO; Density Functional (DFT) at B3LYP Hartreefock methods (HF) with 6-311++G (d,p) level. The energies geometric parameters Kaempferol...

Phenylalanine (Phe) is the most reliable indicator for the diagnosis of phenylketonuria (PKU). The purpose of this study is to establish a reliable and quick method for the assignment of Phe in peripheral capillary blood from newborns and children by high performance liquid chromatography with ultraviolet detection (HPLC-UV). PKU is an inborn error of metabolism characterized by the inability o...

Biosynthetic routes based on cost-efficient, eco-friendly, and sustainable platforms for compounds such as styrene are urgently needed. The biosynthesis of from L-phenylalanine (L-Phe) via trans-cinnamate has long been established, but toxicity limits yields. This study demonstrates that whole-cell cascade biotransformation employing an E. coli consortium expressing respectively phenylalanine a...

Adsorption of phenolic amino acids, such as phenylalanine and tyrosine, is quite relevant for the production of protein hydrolysates used as dietary formulations for patients suffering from congenital disorders of amino acid metabolism, such as phenylketonuria. In this study, an adsorbent prepared from corn cobs was evaluated for the removal of tyrosine (Tyr) from both a single component soluti...

A series of dipeptide ionic liquids (ILs) with l-phenylalanine and l-alanine fragments in structure were synthesized their possible degradation pathways analyzed. Based on this analysis, potential transformation products (PTPs) proposed synthesized. All these compounds (25 total) went through microbial toxicity screening aerobic biodegradation testing. Obtained results demonstrated that by inve...

Higher serum neopterin is associated with increased mortality in patients with coronary artery disease (CAD). Preferentially Th1-type cytokine interferon- γ stimulates neopterin production by GTP cychlohydrolase I (GCH-I) in parallel in monocyte-derived macrophages and dendritic cells. In other cells, activation of GCH-I leads to the formation of 5,6,7,8-tetrahydrobiopterin (BH4), the necessary...