نتایج جستجو برای: phenylalanine
تعداد نتایج: 15351 فیلتر نتایج به سال:
Phenylketonuria (PKU) is an inborn error in phenylalanine metabolism due to deficiency of the enzyme, phenylalanine hydroxylase (PAH). Treatment includes restriction of dietary phenylalanine, and in some individuals, supplementation with the PAH cofactor, tetrahydrobiopterin (sapropterin dihydrochloride). A survey was conducted among patients with PKU who had been prescribed sapropterin to asse...
The Bacillus subtilis anti-TRAP protein regulates the ability of the tryptophan-activated TRAP protein to bind to trp operon leader RNA and promote transcription termination. AT synthesis is regulated both transcriptionally and translationally by uncharged tRNA(Trp). In this study, we examined the roles of AT synthesis and tRNA(Trp) charging in mediating physiological responses to tryptophan st...
A method was developed to study the unsupplemented phenylalanine hydroxylase system in rat liver slices. All of the components of the systemtetrahydrobiopterin, dihydropteridine reductase, and the hydroxylase itself-are present under conditions which should be representative of the actual physiological state of the animal. The properties of the system in liver slices have been compared to those...
It has previously been established that the chick requires phenylalanine in its diet (1,2) as do the rat (3) and mouse (4), but for none of these animals is tyrosine an essential dietary constituent (2-6). Moss and Schoenheimer showed (‘i), by isotope tracer methods, that an appreciable amount of phenylalanine is converted to tyrosine in the rat even when a large amount of tyrosine is present i...
Phenylketonuria (PKU) is a metabolic disorder, in which loss of phenylalanine hydroxylase activity results in neurotoxic levels of phenylalanine. We used the Pah(enu2/enu2) PKU mouse model in short- and long-term studies of enzyme substitution therapy with PEGylated phenylalanine ammonia lyase (PEG-PAL conjugates) from 4 different species. The most therapeutically effective PAL (Av, Anabaena va...
Phenylalanine loading has been proposed as a diagnostic test for autosomal dominant DRD (dopa-responsive dystonia), and recently, a phenylalanine/tyrosine (phe/tyr) ratio of 7.5 after 4 h was reported as diagnostic of DRD. To test the utility of this test in another sample with DRD, we administered an oral challenge of phenylalanine (100 mg/kg) to 11 individuals with DRD and one non-manifesting...
Case Report A female infant weighing 3370 g was born by normal delivery at 39 weeks gestation to parents who believed that they were distantly related. They had one other child, a female, aged five years who was alive and well. There were no perinatal problems but an abnormal phenylalanine result from routine neonatal Guthrie card screening was confirmed by a plasma phenylalanine concentration ...
Recent clinical studies revealed increased phenylalanine levels and phenylalanine to tyrosine ratios in patients suffering from infection, inflammation and general immune activity. These data implicated down-regulation of activity of phenylalanine hydroxylase by oxidative stress upon in vivo immune activation. Though the structural damage of oxidative stress is expected to be comparably small, ...
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