Role of Phenylalanine and Its Metabolites in Health and Neurological Disorders [PDF]
, 2020 Phenylalanine, an amino acid, is a “building block” of protein. Phenylalanine is a component of food sources and also derived through supplementation. In current treatment, phenylalanine is prescribed as anti-depressant agent.
Akram, Muhammad +6 more
core +2 more sources
Phenylalanine Hydroxylase Misfolding and Pharmacological Chaperones [PDF]
Current Topics in Medicinal Chemistry, 2013 Phenylketonuria (PKU) is a loss-of-function inborn error of metabolism. As many other inherited diseases the main pathologic mechanism in PKU is an enhanced tendency of the mutant phenylalanine hydroxylase (PAH) to misfold and undergo ubiquitin-dependent degradation. Recent alternative approaches with therapeutic potential for PKU aim at correcting the
Oscar Aubi +2 more
openaire +3 more sources
Efficient treatment of molecular excitations in the liquid phase environment via stochastic many-body theory [PDF]
, 2021 Accurate predictions of charge excitation energies of molecules in the disordered condensed phase are central to the chemical reactivity, stability, and optoelectronic properties of molecules and critically depend on the specific environment. Herein, we develop a stochastic GW method for calculating these charge excitation energies.
arxiv +1 more source
Association of immune response with efficacy and safety outcomes in adults with phenylketonuria administered pegvaliase in phase 3 clinical trials [PDF]
, 2018 Background: This study assessed the immunogenicity of pegvaliase (recombinant Anabaena variabilis phenylalanine [Phe] ammonia lyase [PAL] conjugated with polyethylene glycol [PEG]) treatment in adults with phenylketonuria (PKU) and its impact on safety ...
Atkinson, John P +14 more
core +2 more sources
Phenylalanine binding is linked to dimerization of the regulatory domain of phenylalanine hydroxylase. [PDF]
Biochemistry, 2014 Analytical ultracentrifugation has been used to analyze the oligomeric structure of the isolated regulatory domain of phenylalanine hydroxylase. The protein exhibits a monomer–dimer equilibrium with a dissociation constant of ∼46 μM; this value is ...
Zhang S, Roberts KM, Fitzpatrick PF.
europepmc +2 more sources
Saccadic reaction time and ocular findings in phenylketonuria [PDF]
, 2020 Background Phenylketonuria (PKU) is an inherited metabolic disorder characterized by reduced activity of phenylalanine hydroxylase resulting in elevated blood phenylalanine (Phe) concentration.
Hennermann, Julia B. +5 more
core +1 more source
Scientific Reports, 2016 Phenylalanine hydroxylase (PAH) is a key tyrosine-biosynthetic enzyme involved in neurological and melanin-associated physiological processes. Despite extensive investigations in holometabolous insects, a PAH contribution to insect embryonic development ...
P. Simonet +9 more
semanticscholar +1 more source
Catching proteins in liquid helium droplets [PDF]
Phys. Rev. Lett. 105, 133402 (2010), 2010 An experimental approach is presented that allows for the incorporation of large mass/charge selected ions in liquid helium droplets. It is demonstrated that droplets can be efficiently doped with a mass/charge selected amino acid as well as with the much bigger m$\approx$12 000 amu protein Cytochrome C in selected charge states.
arxiv +1 more source
Multiclass Semantic Segmentation to Identify Anatomical Sub-Regions of Brain and Measure Neuronal Health in Parkinson's Disease [PDF]
, 2023 Automated segmentation of anatomical sub-regions with high precision has become a necessity to enable the quantification and characterization of cells/ tissues in histology images. Currently, a machine learning model to analyze sub-anatomical regions of the brain to analyze 2D histological images is not available.
arxiv +1 more source
PKU dietary handbook to accompany PKU guidelines [PDF]
, 2020 Background: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine.
Ahring, K. +18 more
core +6 more sources