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*  Patent US4600692 - Immobilized cells for preparing phenylalanine - Google Patents
The preparation of phenylalanine from cinnamic acid using immobilized cells having phenylalanine ammonia lyase activity is also ... A process is disclosed for preparing phenylalanine which comprises contacting phenylpyruvic acid or phenylpyruvate with ... The preparation of phenylalanine from cinnamic acid using immobilized cells having phenylalanine ammonia lyase activity is also ... 16) reviewed the production of phenylalanine from precursor keto acids. He noted a maximum yield of 63.5% phenylalanine was ...
http://www.google.ca/patents/US4600692
*  Low Phenylalanine Diet for Mothers With Phenylketonuria (PKU) - Full Text View - ClinicalTrials.gov
Phenylalanine hydroxylase is an enzyme involved in the metabolism of phenylalanine (Phe). When phenylalanine hydroxylase is ... Low Phenylalanine Diet for Mothers With Phenylketonuria (PKU). The safety and scientific validity of this study is the ... Drug Information available for: Phenylalanine Genetic and Rare Diseases Information Center resources: Phenylketonuria Inborn ... PKU is an autosomal recessive trait caused by the absence of phenylalanine hydroxylase. ...
https://clinicaltrials.gov/show/NCT00065299
*  phenylalanine 2-monooxygenase(EC 1.13.12.9) - Creative Enzymes
L-phenylalanine + O2 + H2O = 3-phenylpyruvic acid + ammonia + H2O2 a reaction simil ... The reaction shown above is about 80% of the reaction catalysed; the remaining 20% is: L-phenylalanine + O2 + H2O = 3- ...
https://www.creative-enzymes.com/product/Phenylalanine-2monooxygenase_10792.html
*  Affinity chromatography and inhibition of chorismate mutase-prephenate dehydrogenase by derivatives of phenylalanine and...
N-benzenesulphonyl-L-phenylalanine and N-benzloxycarbonyl-L-phenylalanine. Consequently two compounds, N-toluene-sulphonyl-L-p- ... Several derivatives of phenylalanine and tyrosine were prepared and tested for inhibition of chorismate mutase-prephenate ... Affinity chromatography and inhibition of chorismate mutase-prephenate dehydrogenase by derivatives of phenylalanine and ... Affinity chromatography and inhibition of chorismate mutase-prephenate dehydrogenase by derivatives of phenylalanine and ...
http://www.biochemj.org/content/165/1/121
*  Adults with late diagnosed PKU and severe challenging behaviour: a randomised placebo-controlled trial of a phenylalanine...
Adults with late diagnosed PKU and severe challenging behaviour: a randomised placebo-controlled trial of a phenylalanine- ... Adults with late diagnosed PKU and severe challenging behaviour: a randomised placebo-controlled trial of a phenylalanine- ... Anecdotal evidence suggests that a diet that lowers blood phenylalanine concentration can have significant benefits upon ... Methods: A prospective double-blind randomised placebo-controlled crossover trial of phenylalanine-restricted diet was ...
http://jnnp.bmj.com/content/80/6/631
*  Phenylalanine Hydroxylase Deficiency (PAH) - Sema4
Phenylalanine Hydroxylase Deficiency (PAH). Phenylalanine hydroxylase deficiency is an autosomal recessive disorder caused by ... Phenylalanine Hydroxylase Deficiency PAH. 1-13 [13] (*29). AR. Ashkenazi Jewish. 1 in 225. 17%. 0.003690036900369. ... Phenylalanine Hydroxylase Deficiency PAH. 1-13 [13] (*29). AR. Sephardic Jewish - Iranian, Bukharian, Kavkazi, Tunisian and ... Phenylalanine Hydroxylase Deficiency PAH. 1-13 [13] (*29). AR. Caucasian. 1 in 50. 75%. 0.0050761421319797. ...
https://sema4genomics.com/products/for-pregnancy/diseases-screened/phenylalanine-hydroxylase-deficiency-2/
*  Responsividade à tetrahidrobiopterina em pacientes com deficiência de fenilalanina hidroxilase
The inclusion criteria were: diagnosis of HPA-PAH; age , 7 years; phenylalanine-restricted diet and phenylalanine (Phe) levels ... Keywords : Phenylketonuria; phenylalanine hydroxylase; biopterin. · abstract in Portuguese · text in English , Portuguese · ... GIUGLIANI, Luciana et al. Tetrahydrobiopterin responsiveness of patients with phenylalanine hydroxylase deficiency. J. Pediatr ... in a sample of Brazilians with hyperphenylalaninemia due to phenylalanine hydroxylase deficiency (HPA-PAH). METHODS: ...
http://www.scielo.br/scielo.php?script=sci_abstract&pid=S0021-75572011000300011&lng=en&nrm=iso&tlng=en
*  Mild phenylketonuria | Genetic and Rare Diseases Information Center (GARD) - an NCATS Program
Treatment is with a diet low in phenylalanine (patients can have up to 400-600 mg/day of phenylalanine).[1][2] Last updated: 12 ... Patients with blood phenylalanine concentrations of 600-1,200 micromol/L are considered to have mild PKU. Clinical signs ... in the PAH gene which result in slightly higher activity of the phenylalanine hydroxylase compared with the classic ... Phenylalanine Hydroxylase Deficiency. GeneReviews. January 31, 2013; http://www.ncbi.nlm.nih.gov/books/NBK1504/. Accessed 12/28 ...
https://rarediseases.info.nih.gov/diseases/10324/benign-hyperphenylalaninemia
*  FDA approves Kuvan for treatment of PKU - Drug Store News
The result is high levels of phenylalanine in the blood. High levels of phenylalanine hydroxylase are toxic to the brain and ... PKU is a genetic disorder in which the enzyme phenylalanine hydroxylase, which helps the human body break down phenylalanine, ... This then leads to an increased breakdown of phenylalanine, resulting in lower levels of phenylalanine in the blood. ... Kuvan works by increasing phenylalanine hydroxylase enzyme activity in PKU patients with some residual phenylalanine ...
http://www.drugstorenews.com/article/fda-approves-kuvan-treatment-pku?ad=pharmacy
*  Phenylketonuria - Zurich Open Repository and Archive
Possible future treatments include enzyme substitution with phenylalanine ammonia lyase, which degrades phenylalanine, and gene ... Possible future treatments include enzyme substitution with phenylalanine ammonia lyase, which degrades phenylalanine, and gene ... and in those patients serves as a useful adjunct to the phenylalanine-restricted diet because it increases phenylalanine ... and in those patients serves as a useful adjunct to the phenylalanine-restricted diet because it increases phenylalanine ...
http://www.zora.uzh.ch/id/eprint/38589/
*  Comparison of Gut Microbial Community in Infants and Toddlers with and without Phenylketonuria
The analysis of the 3-day food record revealed significant differences in protein, phenylalanine and dietary fiber intakes. ... consume low amounts of dietary sources of prebiotics because of their comprehensive dietary restriction of phenylalanine- ...
https://digital.lib.washington.edu/researchworks/handle/1773/26332
*  What Is Biochemistry - Phenylketonuria - Medicalrealm
In normal individual, phenylalanine hydroxylase will convert the phenylalanine into tyrosine. Phenylketonuria may lead to ... Phenylalanine will inhibit amino acid transporter competitively. This will lead to impairment of the protein synthesis, ... Phenylketonuria is also occurs due to the defect in the tetrahydrobiopterin which is a form of cofactor for phenylalanine ... Phenylketonuria occurs due to mutation and impairment of the function of phenylalanine hydroxylase enzyme.. ...
http://www.medicalrealm.net/what-is-biochemistry---phenylketonuria.html
*  7-Substituted pterins in humans with suspected pterin-4a-carbinolamine dehydratase deficiency : mechanism of formation via non...
We have now obtained identical results using human phenylalanine hydroxylase. The identity of the pterin formed in vitro and ... and are formed during hydroxylation of phenylalanine to tyrosine with rat liver dehydratase-free phenylalanine hydroxylase.,br ... and are formed during hydroxylation of phenylalanine to tyrosine with rat liver dehydratase-free phenylalanine hydroxylase. ... in the phenylalanine hydroxylase reaction. For the first time, the formation of 7-substituted pterins from their 6-substituted ...
https://kops.uni-konstanz.de/handle/123456789/7681
*  Phenylketonuria PKU - Essay about Health, Medicine
Even though placing the baby on a phenylalanine-restricted diet with in the first few weeks of life, and maintaining good diet ... Phenylalanine, Inborn error of metabolism Essays Related to Phenylketonuria PKU. Abortion. abortion Click Here to Visit our ... Health, Medicine, Clinical medicine, Intellectual disability, Rare diseases, Phenylketonuria, RTT, Metabolism, Phenylalanine ... PAH is responsible for converting excess phenylalanine into tyrosine and other useful amino acids. Tyrosine is an essential ...
https://eduessays.com/essays/phenylketonuria-pku
*  PKU (Phenylketonuria) - Diseases & Conditions | Med Log - Your Everyday Health Blog
If you or your child is following a low-phenylalanine diet, you'll need to keep records of the amount of phenylalanine eaten ... Because regular infant formula and breast milk contain phenylalanine, babies with PKU instead need to consume a phenylalanine- ... Follow a low-phenylalanine diet. Women with PKU can prevent birth defects by sticking to - or returning to - a low- ... Here are some ideas for serving up three daily meals and a snack with low amounts of phenylalanine:. *Breakfast. In the morning ...
http://medlog.info/pku-phenylketonuria-diseases-conditions-med-log/
*  Evaluating cardiac gene expression in maternal phenylketonuria offspring
It was hypothesized that an association exists between maternal blood phenylalanine (Phe) levels and expression levels of ...
https://soar.wichita.edu/handle/10057/356
*  Phenylalanine | Encyclopedia.com
phenylalanine (fĕn´əlăl´ənēn´), organic compound, one of the 22 α-amino acids [1] commonly found in animal proteins. Only the l ... phenylalanine An aromatic, non-polar amino acid.. Cite this article Pick a style below, and copy the text for your bibliography ... phenylalanine (fĕn´əlăl´ənēn´), organic compound, one of the 22 α-amino acids commonly found in animal proteins. Only the l- ... phenylalanine (fee-nyl-al-ă-neen) n. an essential amino acid that is readily converted to tyrosine. Blockade of this metabolic ...
https://www.encyclopedia.com/science-and-technology/biochemistry/biochemistry/phenylalanine
*  L-phenylalanine (CHEBI:17295)
... is enantiomer of D-phenylalanine (CHEBI:16998) L-phenylalanine (CHEBI:17295) is tautomer of L- ... L-phenylalanine (CHEBI:17295) is a phenylalanine (CHEBI:28044) L-phenylalanine (CHEBI:17295) is a proteinogenic amino acid ( ... D-phenylalanine (CHEBI:16998) is enantiomer of L-phenylalanine (CHEBI:17295). L-phenylalanine residue (CHEBI:29997) is ... L-phenylalanine (CHEBI:17295) has role plant metabolite (CHEBI:76924) L-phenylalanine (CHEBI:17295) is a L-α-amino acid (CHEBI: ...
https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:17295
*  Phenylalanine | chemical compound | Britannica.com
Phenylalanine, an amino acid present in the mixture obtained upon hydrolysis of common proteins. Human hemoglobin (the oxygen- ... to lack of the enzyme phenylalanine hydroxylase, so that phenylalanine is converted not to tyrosine but to phenylpyruvic acid. ... Phenylalanine, an amino acid present in the mixture obtained upon hydrolysis of common proteins. Human hemoglobin (the oxygen- ... More About Phenylalanine. 11 references found in Britannica articles. Assorted References. *amino acids* In human nutrition: ...
https://www.britannica.com/science/phenylalanine
*  Phenylalanine
Categories Supplements Amino Acids Phenylalanine Categories Health Topics Depression Phenylalanine Categories Health Topics ...
https://www.iherb.com/c/Phenylalanine
*  L-phenylalanine zwitterion (CHEBI:58095)
... is enantiomer of D-phenylalanine zwitterion (CHEBI:57981) L-phenylalanine zwitterion ( ... D-phenylalanine zwitterion (CHEBI:57981) is enantiomer of L-phenylalanine zwitterion (CHEBI:58095). L-phenylalanine (CHEBI: ... CHEBI:58095 - L-phenylalanine zwitterion. Main. ChEBI Ontology. Automatic Xrefs. Reactions. Pathways. Models. ... An amino acid zwitterion arising from transfer of a proton from the carboxy to the amino group of L-phenylalanine; major ...
https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:58095
*  phenylalanine
A definition of the medical term "phenylalanine" is presented. Phenylalanine is an essential amino acid and is one of the two ... A definition of the term "hyperphenylalaninemia" is presented, which refers to an increased amount of phenylalanine in the ...
http://connection.ebscohost.com/c/reference-entries/21240593/phenylalanine
*  Phenylalanine Hydroxylase Deficiency - GeneReviews® - NCBI Bookshelf
Affected individuals on an unrestricted diet who have phenylalanine levels above normal but below 1200 μmol/L (20 mg/dL) are at ... deficiency results in intolerance to the dietary intake of the essential amino acid phenylalanine and produces a spectrum of ... Phenylalanine-4-hydroxylase. PAH database. Phenylalanine Hydroxylase Gene Locus-Specific Database - PAHvdb. PAH. PAH. ... A plasma phenylalanine concentration persistently above 120 µmol/L (2 mg/dL) and altered ratio of phenylalanine to tyrosine in ...
https://www.ncbi.nlm.nih.gov/books/NBK1504/