Results 51 to 60 of about 165,999 (264)
The Pharmabiotic for Phenylketonuria: Development of a Novel Therapeutic [PDF]
, 2019 Phenylketonuria, now known as phenylalanine hydroxylase (PAH) deficiency, is a genetic disorder of metabolism affecting approximately one in every 15,000 infants born in the United States.
LeBegue, Chloé Elizabeth
core +1 more source
Liver phenylalanine hydroxylase assay [PDF]
Biochemical Medicine, 1976 The first reaction is catalyzed by phenylalanine hydroxylase and the second reaction, which generates the reduced form of pteridine cofactor (biopterin), is catalyzed by dihydropteridine reductase (l-3). A direct assay of phenylalanine hydroxylase can be achieved by supplying optimal concentrations of reduced pteridine cofactor or an analog of the ...
openaire +4 more sources
, 2017 Nearly half of all patients diagnosed with phenylalanine hydroxylase (PAH) deficiency, also known as phenylketonuria, are lost to follow-up (LTFU); most are adults who stopped attending clinic afte...
J. Thomas +5 more
semanticscholar +1 more source
Integrated molar absorptivity of mid- and far-infrared spectra of glycine and other selected amino acids [PDF]
, 2020 A selection of five proteinogenic amino acids, namely glycine, isoleucine, phenylalanine, tyrosine and tryptophan were studied in the mid-infrared and in the far-infrared with the purpose to facilitate the search and identification of these astrobiological and astrochemical relevant molecules in space environments.
arxiv +1 more source
A unique dual activity amino acid hydroxylase in Toxoplasma gondii [PDF]
, 2009 The genome of the protozoan parasite Toxoplasma gondii was found to contain two genes encoding tyrosine hydroxylase; that produces L-DOPA. The encoded enzymes metabolize phenylalanine as well as tyrosine with substrate preference for tyrosine.
Gaskell, EA +4 more
core +1 more source
Biochemistry, 2015 Liver phenylalanine hydroxylase is allosterically activated by phenylalanine. The structural changes that accompany activation have not been identified, but recent studies of the effects of phenylalanine on the isolated regulatory domain of the enzyme ...
Shengnan Zhang, A. Hinck, P. Fitzpatrick
semanticscholar +1 more source
Dynamic regulation of phenylalanine hydroxylase
Pteridines, 2014 Abstract Phenylalanine hydroxylase (PAH) is the key enzyme in phenylalanine metabolism, catalyzing its oxidative breakdown to tyrosine. Its function in the committed step of amino acid metabolism requires strict regulation. Thus, several regulatory mechanisms are central for an understanding of PAH at the atomistic level.
Klaus R. Liedl +2 more
openaire +3 more sources
Recommendations for the nutrition management of phenylalanine hydroxylase deficiency
Genetics in Medicine, 2014 The effectiveness of a phenylalanine-restricted diet to improve the outcome of individuals with phenylalanine hydroxylase deficiency (OMIM no. 261600) has been recognized since the first patients were treated 60 years ago.
Rani H. Singh +11 more
semanticscholar +1 more source
pH Dependent surface enhanced Raman study of Phe + Ag Complex and DFT calculations for spectral analysis [PDF]
, 2006 Surface enhanced Raman spectra of Phenylalanine (Phe) in Ag colloidal solution have been recorded for Phe solutions of different pH. Spectral line-shape analyses of the enhanced modes, at 1005, 1380 and 1582 cm-1, between pH 4.5 and 10.5, have been carried out. The variation of spectral line-width with pH reveals two possible mechanisms in solution: (i)
arxiv +1 more source
Activation of Phenylalanine Hydroxylase by Phenylalanine Does Not Require Binding in the Active Site
Biochemistry, 2014 Phenylalanine hydroxylase (PheH), a liver enzyme that catalyzes the hydroxylation of excess phenylalanine in the diet to tyrosine, is activated by phenylalanine. The lack of activity at low levels of phenylalanine has been attributed to the N-terminus of
K. Roberts +3 more
semanticscholar +1 more source