Summary: An essential aromatic amino acid that is a precursor of MELANIN; DOPAMINE; noradrenalin (NOREPINEPHRINE), and THYROXINE.

Top Publications

  1. pmc Occurrence of the leucine-to-phenylalanine knockdown resistance (kdr) mutation in Anopheles arabiensis populations in Tanzania, detected by a simplified high-throughput SSOP-ELISA method
    Manisha A Kulkarni
    Department of Natural Resource Sciences, McGill University, Macdonald Campus, Ste Anne de Bellevue, Quebec H9X 3V9, Canada
    Malar J 5:56. 2006
  2. ncbi A modern view of phenylalanine ammonia lyase
    M Jason MacDonald
    Department of Chemistry, Cape Breton University, 1250 Grand Lake Road, Sydney, NS B1P 6L2, Canada
    Biochem Cell Biol 85:273-82. 2007
  3. pmc Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
    Adrian W R Serohijos
    Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, NC 27599, USA
    Proc Natl Acad Sci U S A 105:3256-61. 2008
  4. pmc A bimodal distribution of two distinct categories of intrinsically disordered structures with separate functions in FG nucleoporins
    Justin Yamada
    Department of Molecular, Cell, and Developmental Biology, University of California, Santa Cruz, California 95064, USA
    Mol Cell Proteomics 9:2205-24. 2010
  5. ncbi A phenylalanine in DGAT is a key determinant of oil content and composition in maize
    Peizhong Zheng
    Pioneer Hi Bred International Inc, A DuPont Company, 7300 NW 62nd Avenue, PO Box 1004, Johnston, Iowa 50131, USA
    Nat Genet 40:367-72. 2008
  6. ncbi Tyrosine, phenylalanine, and catecholamine synthesis and function in the brain
    John D Fernstrom
    Department of Psychiatry, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
    J Nutr 137:1539S-1547S; discussion 1548S. 2007
  7. ncbi Phenylalanine hydroxylase deficiency
    John J Mitchell
    Department of Medical Genetics, McGill University Health Center, Montreal, Canada
    Genet Med 13:697-707. 2011
  8. pmc The permeability barrier of nuclear pore complexes appears to operate via hydrophobic exclusion
    Katharina Ribbeck
    ZMBH, INF 282, D 69120 Heidelberg, Germany
    EMBO J 21:2664-71. 2002
  9. ncbi Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria
    Lihi Adler-Abramovich
    Department of Molecular Microbiology and Biotechnology, George S Wise Faculty of Life Sciences, Tel Aviv University, Tel Aviv, Israel
    Nat Chem Biol 8:701-6. 2012
  10. ncbi A Myb transcription factor regulates genes of the phenylalanine pathway in maritime pine
    Blanca Craven-Bartle
    Departamento de Biologia Molecular y Bioquimica, Facultad de Ciencias, Campus Universitario de Teatinos, Universidad de Malaga, 29071 Malaga, Spain
    Plant J 74:755-66. 2013

Detail Information

Publications351 found, 100 shown here

  1. pmc Occurrence of the leucine-to-phenylalanine knockdown resistance (kdr) mutation in Anopheles arabiensis populations in Tanzania, detected by a simplified high-throughput SSOP-ELISA method
    Manisha A Kulkarni
    Department of Natural Resource Sciences, McGill University, Macdonald Campus, Ste Anne de Bellevue, Quebec H9X 3V9, Canada
    Malar J 5:56. 2006
    ..Knockdown resistance (kdr) confers resistance to the pyrethroid class of insecticides with cross-resistance to DDT through single nucleotide polymorphisms (SNPs) in the voltage-gated sodium channel gene...
  2. ncbi A modern view of phenylalanine ammonia lyase
    M Jason MacDonald
    Department of Chemistry, Cape Breton University, 1250 Grand Lake Road, Sydney, NS B1P 6L2, Canada
    Biochem Cell Biol 85:273-82. 2007
    b>Phenylalanine ammonia lyase (PAL; E.C., which catalyses the biotransformation of L-phenylalanine to trans-cinnamic acid and ammonia, was first described in 1961 by Koukol and Conn...
  3. pmc Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
    Adrian W R Serohijos
    Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, NC 27599, USA
    Proc Natl Acad Sci U S A 105:3256-61. 2008
    Deletion of phenylalanine-508 (Phe-508) from the N-terminal nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFTR), a member of the ATP-binding cassette (ABC) transporter family, disrupts both ..
  4. pmc A bimodal distribution of two distinct categories of intrinsically disordered structures with separate functions in FG nucleoporins
    Justin Yamada
    Department of Molecular, Cell, and Developmental Biology, University of California, Santa Cruz, California 95064, USA
    Mol Cell Proteomics 9:2205-24. 2010
    ..Their gate is formed by nucleoporins containing large intrinsically disordered domains with multiple phenylalanine-glycine repeats (FG domains)...
  5. ncbi A phenylalanine in DGAT is a key determinant of oil content and composition in maize
    Peizhong Zheng
    Pioneer Hi Bred International Inc, A DuPont Company, 7300 NW 62nd Avenue, PO Box 1004, Johnston, Iowa 50131, USA
    Nat Genet 40:367-72. 2008
    ..We further show that a phenylalanine insertion in DGAT1-2 at position 469 (F469) is responsible for the increased oil and oleic-acid contents...
  6. ncbi Tyrosine, phenylalanine, and catecholamine synthesis and function in the brain
    John D Fernstrom
    Department of Psychiatry, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
    J Nutr 137:1539S-1547S; discussion 1548S. 2007
    ..This review focuses on Tyr and phenylalanine (Phe)...
  7. ncbi Phenylalanine hydroxylase deficiency
    John J Mitchell
    Department of Medical Genetics, McGill University Health Center, Montreal, Canada
    Genet Med 13:697-707. 2011
    b>Phenylalanine hydroxylase deficiency is an autosomal recessive disorder that results in intolerance to the dietary intake of the essential amino acid phenylalanine. It occurs in approximately 1:15,000 individuals...
  8. pmc The permeability barrier of nuclear pore complexes appears to operate via hydrophobic exclusion
    Katharina Ribbeck
    ZMBH, INF 282, D 69120 Heidelberg, Germany
    EMBO J 21:2664-71. 2002
    ..causes a reversible collapse of the permeability barrier of NPCs, which is consistent with the assumption that the barrier is formed by phenylalanine-rich nucleoporin repeats that attract each other through hydrophobic interactions.
  9. ncbi Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria
    Lihi Adler-Abramovich
    Department of Molecular Microbiology and Biotechnology, George S Wise Faculty of Life Sciences, Tel Aviv University, Tel Aviv, Israel
    Nat Chem Biol 8:701-6. 2012
    Phenylketonuria (PKU) is characterized by phenylalanine accumulation and progressive mental retardation caused by an unknown mechanism...
  10. ncbi A Myb transcription factor regulates genes of the phenylalanine pathway in maritime pine
    Blanca Craven-Bartle
    Departamento de Biologia Molecular y Bioquimica, Facultad de Ciencias, Campus Universitario de Teatinos, Universidad de Malaga, 29071 Malaga, Spain
    Plant J 74:755-66. 2013
    ..This crucial metabolic pathway is finely regulated, primarily through transcriptional control, and because phenylalanine is the precursor for phenylpropanoid biosynthesis, the precise regulation of phenylalanine synthesis and use ..
  11. ncbi The -4 phenylalanine is required for substrate ubiquitination catalyzed by HECT ubiquitin ligases
    Catherine Salvat
    Institute for Cellular and Molecular Biology, Section of Molecular Genetics and Microbiology, University of Texas at Austin, Austin, Texas 78712, USA
    J Biol Chem 279:18935-43. 2004
    ..We report the identification of a determinant that is specifically required for the last step of this cycle, a phenylalanine residue located four amino acids from the C terminus of most HECT domains, referred to here as the -4F...
  12. ncbi Mutation of tyrosine 332 to phenylalanine converts dopa decarboxylase into a decarboxylation-dependent oxidative deaminase
    Mariarita Bertoldi
    Dipartimento di Scienze Neurologiche e della Visione, Sezione di Chimica Biologica, Facolta di Medicina e Chirurgia, Universita degli Studi di Verona, Strada Le Grazie, 8, 37134 Verona, Italy
    J Biol Chem 277:36357-62. 2002
    ..The function of tyrosine 332 has been investigated by substituting it with phenylalanine. Y332F displays coenzyme content and spectroscopic features identical to those of the wild type...
  13. pmc Adaptation of phenylalanine and tyrosine catabolic pathway to hibernation in bats
    Yi Hsuan Pan
    Laboratory of Molecular Ecology and Evolution, Institute for Advanced Studies in Multidisciplinary Science and Technology, East China Normal University, Shanghai, China
    PLoS ONE 8:e62039. 2013
    ..of amino acid preservation, we found evidence of amino acid metabolism as three of five key enzymes, including phenylalanine hydroxylase (PAH), homogentisate 1,2-dioxygenase (HGD), fumarylacetoacetase (FAH), involved in phenylalanine ..
  14. ncbi High precision measurement and fragmentation analysis for metabolite identification
    Madalina Oppermann
    Thermo Fisher Scientific, Kungens Kurva, Sweden
    Methods Mol Biol 860:145-56. 2012
    ..Unambiguous identification of four analytes (citric acid, chlorogenic acid, phenylalanine, and UDP-D: -glucose) is used to validate the performance of the different MS/MS fragmentation regimes...
  15. ncbi A novel ensemble-based scoring and search algorithm for protein redesign and its application to modify the substrate specificity of the gramicidin synthetase a phenylalanine adenylation enzyme
    Ryan H Lilien
    Computer Science Department, Dartmouth College, Hanover, NH 03755, USA
    J Comput Biol 12:740-61. 2005
    ..Our ensemble-based algorithm, which flexibly models both protein and ligand using rotamer-based partition functions, has application in enzyme redesign, the prediction of protein-ligand binding, and computer-aided drug design...
  16. ncbi The global gene expression response of Escherichia coli to L-phenylalanine
    T Polen
    Institut für Biotechnologie 1, Forschungszentrum Julich, D 52425 Julich, Germany
    J Biotechnol 115:221-37. 2005
    ..the global gene expression changes of Escherichia coli due to the presence of different concentrations of phenylalanine or shikimate in the growth medium. The response to 0...
  17. pmc Non-physiological amino acid (NPAA) therapy targeting brain phenylalanine reduction: pilot studies in PAHENU2 mice
    Kara R Vogel
    Section of Clinical Pharmacology, College of Pharmacy, Washington State University, Pullman, WA 99164 6510, USA
    J Inherit Metab Dis 36:513-23. 2013
    ..Peripheral accumulation of one LNAA (e.g., phenylalanine (phe) in PKU) is predicted to increase uptake of the offending amino acid to the detriment of others, ..
  18. pmc Site-directed mutagenesis reveals putative substrate binding residues in the Escherichia coli RND efflux pump AcrB
    Jürgen A Bohnert
    Center for Infectious Diseases and Travel Medicine, University Hospital, Department of Medicine, Albert Ludwigs University, Freiburg, Germany
    J Bacteriol 190:8225-9. 2008
    ..efflux pump protein AcrB has recently been cocrystallized with various substrates, suggesting that there is a phenylalanine-rich binding site around F178 and F615...
  19. ncbi Mechanisms underlying molecularly imprinted polymer molecular memory and the role of crosslinker: resolving debate on the nature of template recognition in phenylalanine anilide imprinted polymers
    Gustaf D Olsson
    Bioorganic and Biophysical Chemistry Laboratory, School of Natural Sciences, Linnaeus University, SE 391 82 Kalmar, Sweden
    J Mol Recognit 25:69-73. 2012
    A series of molecular dynamics simulations of prepolymerization mixtures for phenylalanine anilide imprinted co-(ethylene glycol dimethacrylate-methacrylic acid) molecularly imprinted polymers have been employed to investigate the ..
  20. pmc Adeno-associated virus serotype 6 capsid tyrosine-to-phenylalanine mutations improve gene transfer to skeletal muscle
    Chunping Qiao
    Division of Molecular Pharmaceutics, University of North Carolina Eshelman School of Pharmacy, Chapel Hill, NC 27599, USA
    Hum Gene Ther 21:1343-8. 2010
    ..Here, we hypothesize that AAV6 transduction efficiency can be further enhanced by mutating surface Y to phenylalanine (F)...
  21. ncbi Structural characterization of nanoscale meshworks within a nucleoporin FG hydrogel
    Marcel Petri
    Research Group of Structural Dynamics of Bio chemical Systems, Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
    Biomacromolecules 13:1882-9. 2012
    ..It consists of phenylalanine-glycine (FG) repeat domains apparently organized as an FG hydrogel...
  22. ncbi Evidence that phenylalanine 69 in Escherichia coli RuvC resolvase forms a stacking interaction during binding and destabilization of a Holliday junction DNA substrate
    M Yoshikawa
    Department of Molecular Microbiology, Research Institute for Microbial Diseases, Osaka University, and PRESTO, Japan Science and Technology Corporation, Suita, Osaka, 565 0871, Japan
    J Biol Chem 276:10432-6. 2001
    ..This study examines the phenotype of RuvC mutants with amino acid substitutions at phenylalanine 69 (F69L, F69Y, F69W, and F69A), a catalytically important residue that faces the catalytic center of the ..
  23. ncbi Do adult patients with phenylketonuria improve their quality of life after introduction/resumption of a phenylalanine-restricted diet?
    R Gassió
    PKU Follow up Unit, Department of Neurology, Hospital Sant Joan de Deu, Universitat de Barcelona, Spain
    Acta Paediatr 92:1474-8. 2003
    ..To evaluate the possible influence of dietary treatment on the quality of life of adult patients with PKU (phenylketonuria) following late introduction or resumption of a Phe-restricted diet...
  24. pmc A moderate serving of high-quality protein maximally stimulates skeletal muscle protein synthesis in young and elderly subjects
    T Brock Symons
    Graduate Center for Gerontology, University of Kentucky, Lexington, USA
    J Am Diet Assoc 109:1582-6. 2009
    ..08 mumol/kg/min) of L-[ring-(13)C(6)] phenylalanine in healthy young (n=17; 34+/-3 years) and elderly (n=17; 68+/-2 years) individuals...
  25. pmc Identification of genes in the phenylalanine metabolic pathway by ectopic expression of a MYB transcription factor in tomato fruit
    Valeriano Dal Cin
    University of Florida, Horticultural Sciences, Gainesville, Florida 32611 0690, USA
    Plant Cell 23:2738-53. 2011
    ..However, while increased levels of these human nutrition-related phenylpropanoids may be desirable, there were no increases in levels of Phe-derived flavor volatiles...
  26. ncbi Phenylalanine loading as a diagnostic test for DRD: interpreting the utility of the test
    R Saunders-Pullman
    Department of Neurology, Beth Israel Medical Center, New York, NY, USA
    Mol Genet Metab 83:207-12. 2004
    b>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...
  27. ncbi Living with phenylketonuria: perspectives of patients and their families
    C Bilginsoy
    Department of Economics, University of Utah, Salt Lake City, Utah 84112, USA
    J Inherit Metab Dis 28:639-49. 2005
    ..b>Phenylalanine test results were regarded as the principal signal for the need for dietary adjustment...
  28. ncbi A survey of aspartate-phenylalanine and glutamate-phenylalanine interactions in the protein data bank: searching for anion-π pairs
    Vivek Philip
    Department of Biochemistry and Cellular and Molecular Biology, University of Tennessee, Knoxville, Tennessee 37996 0840, United States
    Biochemistry 50:2939-50. 2011
    ..the role of anion-π interactions in stabilizing protein structure, we analyzed pairwise interactions between phenylalanine (Phe) and the anionic amino acids, aspartate (Asp) and glutamate (Glu)...
  29. pmc A rationally designed pyrrolysyl-tRNA synthetase mutant with a broad substrate spectrum
    Yane Shih Wang
    Department of Chemistry, Texas A and M University, College Station, Texas 77843, USA
    J Am Chem Soc 134:2950-3. 2012
    ..synthetase mutant N346A/C348A has been successfully used for the genetic incorporation of a variety of phenylalanine derivatives with large para substituents into superfolder green fluorescent protein at an amber mutation site ..
  30. ncbi Biosynthetic 13C labeling of aromatic side chains in proteins for NMR relaxation measurements
    Kaare Teilum
    Department of Biophysical Chemistry, Lund University, Sweden
    J Am Chem Soc 128:2506-7. 2006
    ..We validated the method by acquiring 13C rotating-frame relaxation dispersion data on the E140Q mutant of the C-terminal domain of calmodulin, which reveal conformational exchange dynamics with a time constant of 71 mus for Y138...
  31. ncbi Role of phenylalanine B10 in plant nonsymbiotic hemoglobins
    Benoit J Smagghe
    Department of Biochemistry, Biophysics, and Molecular Biology, Iowa State University, Ames, Iowa 50011, USA
    Biochemistry 45:9735-45. 2006
    ..This work presents a structural mechanism for regulating reversible intramolecular coordination in plant hxHbs...
  32. pmc Physiological characterization of the ARO10-dependent, broad-substrate-specificity 2-oxo acid decarboxylase activity of Saccharomyces cerevisiae
    Zeynep Vuralhan
    Department of Biotechnology, Delft University of Technology, Julianalaan 67, 2628 BC Delft, The Netherlands
    Appl Environ Microbiol 71:3276-84. 2005
    ..PK113-7D were grown with different nitrogen sources. Cultures grown with phenylalanine, leucine, or methionine as a nitrogen source contained high levels of the corresponding fusel alcohols and ..
  33. pmc Intramolecular cohesion of coils mediated by phenylalanine--glycine motifs in the natively unfolded domain of a nucleoporin
    V V Krishnan
    Department of Applied Science, University of California Davis, Davis, California, United States of America
    PLoS Comput Biol 4:e1000145. 2008
    ..a size-selective gate formed by a family of NPC proteins that feature large, natively unfolded domains with phenylalanine-glycine repeats (FG domains)...
  34. ncbi Characterization of white matter alterations in phenylketonuria by magnetic resonance relaxometry and diffusion tensor imaging
    Peter Vermathen
    Department Clinical Research, Unit for MR Spectroscopy and Methodology, University Berne, Bern, Switzerland
    Magn Reson Med 58:1145-56. 2007
    ..ADC values in lesions and in the corpus callosum correlated negatively with blood and brain phenylalanine (Phe) concentrations. Intramyelinic edema combined with vacuolization is a likely cause of the WM alterations...
  35. pmc The glycine-phenylalanine-rich region determines the specificity of the yeast Hsp40 Sis1
    W Yan
    Department of Biomolecular Chemistry, University of Wisconsin Madison, Madison, Wisconsin 53706, USA
    Mol Cell Biol 19:7751-8. 1999
    ..231 amino acids but retaining the N-terminal 121 amino acids encompassing the J domain and the glycine-phenylalanine-rich (G-F) region, was able to rescue the inviability of a Deltasis1 strain...
  36. ncbi Suboptimal outcomes in patients with PKU treated early with diet alone: revisiting the evidence
    G M Enns
    Division of Medical Genetics, Department of Pediatrics, Lucile Packard Children s Hospital, Stanford University, Stanford, CA 94305 5208, USA
    Mol Genet Metab 101:99-109. 2010
  37. ncbi Molecular basis for class Ib anti-arrhythmic inhibition of cardiac sodium channels
    Stephan A Pless
    Department of Anesthesiology, University of British Columbia, 2350 Health Science Mall, Vancouver, British Columbia, V6T 1Z3 Canada
    Nat Commun 2:351. 2011
    ..distinct effects on the cardiac action potential by binding to a common receptor? Here we use fluorinated phenylalanine derivatives to test whether the electronegative surface potential of aromatic side chains contributes to ..
  38. pmc Addition of the keto functional group to the genetic code of Escherichia coli
    Lei Wang
    Department of Chemistry, University of California, Berkeley 94720, USA
    Proc Natl Acad Sci U S A 100:56-61. 2003
    ..tRNA-synthetase pair that makes possible the efficient incorporation of a keto amino acid, p-acetyl-l-phenylalanine, into proteins in E. coli with high translational fidelity in response to the amber nonsense codon...
  39. pmc Bacterial phenylalanine and phenylacetate catabolic pathway revealed
    R Teufel
    Department of Mikrobiologie, Fakultat fur Biologie, Albert Ludwigs Universitat Freiburg, D 79104 Freiburg, Germany
    Proc Natl Acad Sci U S A 107:14390-5. 2010
    ..g., phenylalanine or styrene) is metabolized by bacteria via phenylacetate...
  40. pmc Theoretical characterization of substrate access/exit channels in the human cytochrome P450 3A4 enzyme: involvement of phenylalanine residues in the gating mechanism
    Dan Fishelovitch
    Department of Human Molecular Genetics and Biochemistry, Sackler Institute of Molecular Medicine, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    J Phys Chem B 113:13018-25. 2009
    ..Most channels consist of one or two pi-stacked phenylalanine residues that serve as gate keepers...
  41. pmc Mutational analysis of the feedback sites of phenylalanine-sensitive 3-deoxy-D-arabino-heptulosonate-7-phosphate synthase of Escherichia coli
    Y Kikuchi
    Central Research Laboratory, Ajinomoto Co, Inc, Kawasaki, Japan
    Appl Environ Microbiol 63:761-2. 1997
    ..encode 3-deoxy-D-arabino-heptulosonate-7-phosphate synthase isozymes that are feedback inhibited by tyrosine, phenylalanine, and tryptophan, respectively...
  42. ncbi Combined ingestion of protein and free leucine with carbohydrate increases postexercise muscle protein synthesis in vivo in male subjects
    Rene Koopman
    Department of Human Biology, Maastricht University, PO Box 616, 6200 MD Maastricht, The Netherlands
    Am J Physiol Endocrinol Metab 288:E645-53. 2005
    ..A primed, continuous infusion of L-[ring-13C6]phenylalanine was applied, with blood samples and muscle biopsies collected to assess fractional synthetic rate (FSR) in the ..
  43. ncbi Plant phenylacetaldehyde synthase is a bifunctional homotetrameric enzyme that catalyzes phenylalanine decarboxylation and oxidation
    Yasuhisa Kaminaga
    Department of Horticulture and Landscape Architecture, Purdue University, West Lafayette, Indiana 47907 2010, USA
    J Biol Chem 281:23357-66. 2006
    ..and shares extensive amino acid identity (approximately 65%) with plant L-tyrosine/3,4-dihydroxy-L-phenylalanine and L-tryptophan decarboxylases. It displays a strict specificity for phenylalanine with an apparent Km of 1...
  44. pmc Fungal metabolic model for human type I hereditary tyrosinaemia
    J M Fernández-Cañón
    Centro de Investigaciones Biologicas, Consejo Superior de Investigaciones Cientificas, Madrid, Spain
    Proc Natl Acad Sci U S A 92:9132-6. 1995
    ..disruption results in secretion of succinylacetone (a diagnostic compound for human type I tyrosinaemia) and phenylalanine toxicity...
  45. ncbi Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrations
    S C J Huijbregts
    Department of Clinical Neuropsychology, Vrije Universiteit Amsterdam, Van der Boechorststraat 1, 1081 BT, Amsterdam, The Netherlands
    Neuropsychologia 40:7-15. 2002
    ..PKU patients with plasma phenylalanine (phe) levels higher than 360 micromol/l at the time of testing exhibited, compared to controls, lower speed of ..
  46. pmc Skeletal muscle protein anabolic response to resistance exercise and essential amino acids is delayed with aging
    Micah J Drummond
    Department of Physical Therapy, Division of Rehabilitation Sciences, University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555 1144, USA
    J Appl Physiol (1985) 104:1452-61. 2008
    ..Notwithstanding, the combination of resistance exercise and EAA ingestion should be a useful strategy to combat sarcopenia...
  47. ncbi A conserved Tyr residue is required for sugar selectivity in a Pol alpha DNA polymerase
    Guangwei Yang
    Department of Molecular Biophysics and Biochemistry, Yale University, 333 Cedar Street, New Haven, Connecticut 06520, USA
    Biochemistry 41:10256-61. 2002
  48. pmc Structures of Escherichia coli DNA mismatch repair enzyme MutS in complex with different mismatches: a common recognition mode for diverse substrates
    Ganesh Natrajan
    Division of Molecular Carcinogenesis, The Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX, Amsterdam, The Netherlands
    Nucleic Acids Res 31:4814-21. 2003
    ..Two residues widely conserved in the MutS family are involved in mismatch recognition. The phenylalanine, Phe 36, is seen stacking on one of the mismatched bases...
  49. ncbi Anthrax toxin: receptor binding, internalization, pore formation, and translocation
    John A T Young
    Infectious Disease Laboratory, The Salk Institute for Biological Studies, La Jolla, California 92037, USA
    Annu Rev Biochem 76:243-65. 2007
  50. pmc Small-molecule CD4 mimics interact with a highly conserved pocket on HIV-1 gp120
    Navid Madani
    Department of Cancer Immunology and AIDS, Dana Farber Cancer Institute, 44 Binney Street, JFB 824, Boston, MA 02115, USA
    Structure 16:1689-701. 2008
  51. ncbi Addition of p-azido-L-phenylalanine to the genetic code of Escherichia coli
    Jason W Chin
    Department of Chemistry and The Skaggs Institute for Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, California 92037, USA
    J Am Chem Soc 124:9026-7. 2002
    ..orthogonal aminoacyl tRNA synthetase/tRNA pair for the in vivo incorporation of a photocrosslinker, p-azido-l-phenylalanine, into proteins in response to the amber codon, TAG...
  52. ncbi Phenylalanine tolerance can already reliably be assessed at the age of 2 years in patients with PKU
    F J van Spronsen
    Department of Pediatrics, Beatrix Children s Hospital, and Center for Liver, Digestive and Metabolic Diseases, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands
    J Inherit Metab Dis 32:27-31. 2009
    The clinical severity of phenylalanine hydroxylase deficiency is usually defined by either pre-treatment phenylalanine (Phe) concentration or Phe tolerance at 5 years of age...
  53. ncbi Kinetic analysis of the inhibition of phenylalanine ammonia-lyase by 2-aminoindan-2-phosphonic acid and other phenylalanine analogues
    Christoph Appert
    Institute of Plant Sciences, Federal Institute of Technology Zurich, CH 8092 Zurich, Switzerland
    Phytochemistry 62:415-22. 2003
    The conformationally restricted phenylalanine analogue 2-aminoindan-2-phosphonic acid (AIP) inhibits phenylalanine ammonia-lyase (PAL) competitively in a time-dependent manner...
  54. ncbi Importance of phenylalanine 107 in agonist recognition by the 5-hydroxytryptamine(3A) receptor
    L J Steward
    Yoshitomi Research Institute of Neuroscience in Glasgow, University of Glasgow, United Kingdom
    Mol Pharmacol 57:1249-55. 2000
    ..This study examines the effects of altering phenylalanine 107 (F107) of the 5-HT(3AL) subunit, obtained from NG108-15 cells, using site-directed mutagenesis...
  55. pmc The conserved phenylalanine in the K+ channel voltage-sensor domain creates a barrier with unidirectional effects
    Christine S Schwaiger
    Science for Life Laboratory and Swedish e Science Research Center, KTH Royal Institute of Technology and Stockholm University, Solna, Sweden
    Biophys J 104:75-84. 2013
    ..This barrier is critical for channel function, and a conserved phenylalanine in segment S2 has previously been identified to be highly sensitive to substitutions...
  56. pmc Using change in plasma phenylalanine concentrations and ability to liberalize diet to classify responsiveness to tetrahydrobiopterin therapy in patients with phenylketonuria
    Rani H Singh
    Department of Human Genetics, Emory University School of Medicine, Decatur, GA 30033, USA
    Mol Genet Metab 104:485-91. 2011
    Tetrahydrobiopterin (BH(4)) responsiveness is currently defined as a decrease in plasma phenylalanine concentrations in patients with phenylketonuria (PKU)...
  57. ncbi Probing phenylalanine/adenine pi-stacking interactions in protein complexes with explicitly correlated and CCSD(T) computations
    Kari L Copeland
    Department of Chemistry and Biochemistry, University of Mississippi, University, Mississippi 38677, USA
    J Phys Chem B 112:14291-5. 2008
    ..The MP2 method overbinds the crystal structures by 2.31 kcal mol (-1) on average compared to 4.50 kcal mol (-1) for the optimized structures...
  58. pmc Identification of a highly conserved valine-glycine-phenylalanine amino acid triplet required for HIV-1 Nef function
    Pieter J Meuwissen
    Department of Clinical Chemistry, Microbiology, and Immunology, Ghent University, Ghent, B 9000, Belgium
    Retrovirology 9:34. 2012
    ..clinical isolates, we identified a dysfunctional HIV group O Nef in which a highly conserved valine-glycine-phenylalanine (VGF) region, which links a preceding acidic cluster with the following proline-rich motif into an amphipathic ..
  59. ncbi L-amino acid transporter-1 and boronophenylalanine-based boron neutron capture therapy of human brain tumors
    Allah Detta
    Department of Neurosurgery, Molecular Neurooncology Laboratory, The University of Birmingham, Queen Elizabeth Hospital Neuroscience Centre, Edgbaston, Birmingham, United Kingdom
    Cancer Res 69:2126-32. 2009
    ..005; n = 4 glioblastoma tumors). This uptake was inhibited by both phenylalanine and 2-aminobicyclo-(2,2,1)-heptane-2-carboxylic acid...
  60. ncbi Phenylalanine activates the mitochondria-mediated apoptosis through the RhoA/Rho-associated kinase pathway in cortical neurons
    Yongjun Zhang
    Xinhua Hospital, Shanghai Institute for Pediatric Research, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China
    Eur J Neurosci 25:1341-8. 2007
    Phenylketonuria (PKU) is caused by deficiency of phenylalanine hydroxylase, resulting in an accumulation of phenylalanine in brain tissue and cerebrospinal fluid of phenylketonuria patients...
  61. ncbi FG-rich repeats of nuclear pore proteins form a three-dimensional meshwork with hydrogel-like properties
    Steffen Frey
    Zentrum für Molekulare Biologie der Universität Heidelberg ZMBH, INF 282, D 69120 Heidelberg, Germany
    Science 314:815-7. 2006
    ..Here, we found that phenylalanine-mediated inter-repeat interactions indeed cross-link FG-repeat domains into elastic and reversible hydrogels...
  62. ncbi Inhibitory control in children with phenylketonuria
    Shawn E Christ
    Department of Psychological Sciences, University of Missouri Columbia, Department of Psychology, Washington University, St Louis, MO 65211, USA
    Dev Neuropsychol 30:845-64. 2006
    ..Findings suggest that the inhibitory deficit associated with PKU is subtle and that inconsistent findings in past studies may be largely due to the insensitivity of experimental manipulations in some tasks...
  63. ncbi Structural basis of autoregulation of phenylalanine hydroxylase
    B Kobe
    St Vincent s Institute of Medical Research, Fitzroy, Victoria, Australia
    Nat Struct Biol 6:442-8. 1999
    b>Phenylalanine hydroxylase converts phenylalanine to tyrosine, a rate-limiting step in phenylalanine catabolism and protein and neurotransmitter biosynthesis...
  64. ncbi Phenylalanine 368 of multidrug resistance-associated protein 4 (MRP4/ABCC4) plays a crucial role in substrate-specific transport activity
    Hanneke G M Wittgen
    Department of Pharmacology and Toxicology, Radboud University Nijmegen Medical Centre, Nijmegen Centre for Molecular Life Sciences, The Netherlands
    Biochem Pharmacol 84:366-73. 2012
    ..They, however, might interact with the substrates during rearrangement of helixes for substrate translocation, funneling the substrates to the exit site in the outward-facing conformation...
  65. pmc Structures of open (R) and close (T) states of prephenate dehydratase (PDT)--implication of allosteric regulation by L-phenylalanine
    Kemin Tan
    Midwest Center for Structural Genomics and Structural Biology Center, Biosciences Division, Building 202, Room A125 9700, S Cass Avenue, Argonne National Laboratory, Argonne, IL 60439, USA
    J Struct Biol 162:94-107. 2008
    The enzyme prephenate dehydratase (PDT) converts prephenate to phenylpyruvate in L-phenylalanine biosynthesis. PDT is allosterically regulated by L-Phe and other amino acids...
  66. ncbi High-level cell-free synthesis yields of proteins containing site-specific non-natural amino acids
    Aaron R Goerke
    Department of Chemical Engineering, Stanford University, Stanford, California 94305, USA
    Biotechnol Bioeng 102:400-16. 2009
    ..up to 400 microg/mL of eCAT109pAz, 660 microg/mL of eDHFR10pAz, and 210 microg/mL of mDHFR31pAz with p-azido-L-phenylalanine (pAz) incorporated site-specifically at the amber nonsense codon...
  67. ncbi Adherence to diet and quality of life in patients with phenylketonuria
    G Cotugno
    Division of Metabolic Diseases, Bambino Gesu Children s Hospital, Rome, Italy
    Acta Paediatr 100:1144-9. 2011
    ..To investigate adherence to dietary treatment and quality of life (QoL) in patients with phenylketonuria (PKU)...
  68. ncbi DNA binding and antimicrobial studies of polymer-copper(II) complexes containing 1,10-phenanthroline and L-phenylalanine ligands
    Rajendran Senthil Kumar
    School of Chemistry, Bharathidasan University, Tiruchirappalli 620 024, Tamil Nadu, India
    Eur J Med Chem 44:1878-83. 2009
    ..4H(2)O (phen=1,10-phenanthroline, l-phe=l-phenylalanine, and BPEI=branched polyethyleneimine), with various amounts of copper(II) chelates in the polymer chain, were ..
  69. ncbi Modeling and optimization of phenylalanine ammonia lyase stabilization in recombinant Escherichia coli for the continuous synthesis of l-phenylalanine on the statistical-based experimental designs
    Bing Zhu Zhang
    Department of Scientific Research, Hebei University of Science and Technology, 70 Yuhua East Road, Shijiazhang 050018, People s Republic of China
    J Agric Food Chem 58:2795-800. 2010
    Some approaches for improving recombinant phenylalanine ammonia lyase (PAL) stability in Escherichia coli during the enzymatic methods of l-phenylalanine (l-Phe) production were developed following preliminary studies by means of ..
  70. pmc C-terminal phenylalanine of bacteriophage T7 single-stranded DNA-binding protein is essential for strand displacement synthesis by T7 DNA polymerase at a nick in DNA
    Sharmistha Ghosh
    Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 284:30339-49. 2009
    ..The acidic C-terminal tail of gp2.5, bearing a C-terminal phenylalanine, physically and functionally interacts with the helicase and DNA polymerase...
  71. ncbi Accumulation of the hydroxyl free radical markers meta-, ortho-tyrosine and DOPA in cataractous lenses is accompanied by a lower protein and phenylalanine content of the water-soluble phase
    Gergo A Molnar
    Second Department of Medicine and Nephrological Center, University of Pecs, Faculty of Medicine, Pecs, Hungary
    Free Radic Res 39:1359-66. 2005
    ..In our HPLC analysis, oxidatively modified derivatives of phenylalanine were detected in cataractous samples...
  72. ncbi Neuropsychological outcome of subjects participating in the PKU adult collaborative study: a preliminary review
    V L Brumm
    CHLA Division of Neurology, Keck School of Medicine, University of Southern California, 4650 Sunset Blvd, MS 82, Los Angeles, CA 90027, USA
    J Inherit Metab Dis 27:549-66. 2004
    ..resonance spectroscopy (MRS) to identify: (1) pattern of cognitive dysfunction; (2) effect of high blood phenylalanine (Phe) level at time of cognitive testing; and (3) treatment variables that may be associated with cognitive ..
  73. ncbi In vivo incorporation of an alkyne into proteins in Escherichia coli
    Alexander Deiters
    Department of Chemistry, Skaggs Institute for Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Bioorg Med Chem Lett 15:1521-4. 2005
    ..These evolved tRNA-synthetase pairs were used to site-specifically incorporate an alkynyl group into a protein, which was subsequently conjugated with fluorescent dyes by a [3+2]-cycloaddition reaction under mild reaction conditions...
  74. ncbi Characterization of Ruminococcus albus cellodextrin phosphorylase and identification of a key phenylalanine residue for acceptor specificity and affinity to the phosphate group
    Tatsuya Sawano
    Research Faculty of Agriculture, Hokkaido University, Sapporo, Japan
    FEBS J 280:4463-73. 2013
    ..Furthermore, the F633Y mutant showed 75- and 1100-fold lower apparent Km values for inorganic phosphate and Glc1P, respectively, in phosphorolysis and synthesis of cellotriose...
  75. ncbi Diurnal variation of phenylalanine concentrations in tyrosinaemia type 1: should we be concerned?
    A Daly
    Birmingham Children s Hospital, Steelhouse Lane, Birmingham, UK
    J Hum Nutr Diet 25:111-6. 2012
    Tyrosinaemia type 1 (HT1) is treated with a tyrosine and phenylalanine-restricted diet, amino acids free of phenylalanine and tyrosine, and nitisinone (NTBC)...
  76. pmc A Single phenylalanine residue in the main intracellular loop of α1 γ-aminobutyric acid type A and glycine receptors influences their sensitivity to propofol
    Gustavo Moraga-Cid
    Department of Physiology, University of Concepcion, Concepcion, Chile
    Anesthesiology 115:464-73. 2011
    ..Therefore, the influence of the large intracellular loop in propofol sensitivity of both receptors was explored...
  77. pmc Crystal structure of LAAO from Calloselasma rhodostoma with an L-phenylalanine substrate: insights into structure and mechanism
    Ibrahim M Moustafa
    Department of Chemistry and Biochemistry, University of California, Santa Cruz, Santa Cruz, CA 95064, USA
    J Mol Biol 364:991-1002. 2006
    ..The structure of l-amino acid oxidase with its substrate (L-phenylalanine) has been refined to a resolution of 1.8 A...
  78. ncbi Aromatic amino acid aminotransferase activity and indole-3-acetic acid production by associative nitrogen-fixing bacteria
    Raúl Osvaldo Pedraza
    Centro de investigaciones microbiológicas, Instituto de Ciencias, Universidad Autonoma de Puebla, Apdo Postal 1622 C P 72, 000, Puebla Pue, Mexico
    FEMS Microbiol Lett 233:15-21. 2004
    ..404 micromol min(-1)mg(-1). When analyzed under non-denaturating PAGE conditions; and using tryptophan, phenylalanine, tyrosine, and histidine as substrates Pseudomonas stutzeri A15 showed three isoforms with molecular mass of ..
  79. pmc Identification and characterization of phenylpyruvate decarboxylase genes in Saccharomyces cerevisiae
    Zeynep Vuralhan
    Kluyver Laboratory of Biotechnology, Delft University of Technology, 2628 BC Delft, The Netherlands
    Appl Environ Microbiol 69:4534-41. 2003 functional in Saccharomyces cerevisiae, since during growth in glucose-limited chemostat cultures with phenylalanine as the sole nitrogen source, phenylethanol and phenylacetate were produced in quantities that accounted for ..
  80. ncbi Treatment of multiple sclerosis with lofepramine, L-phenylalanine and vitamin B(12): mechanism of action and clinical importance: roles of the locus coeruleus and central noradrenergic systems
    C Loder
    Med Hypotheses 59:594-602. 2002
    In a randomized, placebo-controlled double-blind trial a combination of lofepramine, phenylalanine and vitamin B(12) was found to be effective in relieving the symptoms of multiple sclerosis (MS)...
  81. ncbi A novel phenylalanine-based targeting signal directs telencephalin to neuronal dendrites
    Sachiko Mitsui
    Laboratory for Neurobiology of Synapse, RIKEN Brain Science Institute, Wako, Saitama 351 0198, Japan
    J Neurosci 25:1122-31. 2005
  82. ncbi Direct observation of the release of phenylalanine from diphenylalanine nanotubes
    Victoria L Sedman
    Department of Molecular Microbiology and Biotechnology, George S Wise Faculty of Life Sciences, Tel Aviv University, Tel Aviv 69978, Israel
    J Am Chem Soc 128:6903-8. 2006
    The core recognition motif of the amyloidogenic beta-amyloid polypeptide is a dipeptide of phenylalanine. This dipeptide readily self-assembles to form discrete, hollow nanotubes with high persistence lengths...
  83. pmc The role of the conserved phenylalanine in the sigma54-interacting GAFTGA motif of bacterial enhancer binding proteins
    Nan Zhang
    Division of Biology, Sir Alexander Fleming Building, and Centre for Molecular Microbiology and Infection, Flowers Building, Imperial College London, London SW7 2AZ, UK
    Nucleic Acids Res 37:5981-92. 2009
    ..We demonstrate that substitution of a highly conserved phenylalanine (F85) residue within the L1 loop GAFTGA motif affects (i) the ATP hydrolysis rate of PspF, demonstrating the ..
  84. pmc Evaluation of orally administered PEGylated phenylalanine ammonia lyase in mice for the treatment of Phenylketonuria
    Christineh N Sarkissian
    Departments of Biology, Human Genetics and Pediatrics, McGill University, and Debelle Laboratory, McGill University Montreal Children s Hospital Research Institute, 2300 Tupper Street, A 717, Montreal, QC, H3H 1P3 Canada
    Mol Genet Metab 104:249-54. 2011
    ..PKU mouse model to study oral enzyme substitution therapy with various chemically modified formulations of phenylalanine ammonia lyase (Av-p.C503S/p.C565S/p.F18A PAL)...
  85. ncbi Melanism in guinea fowl (Numida meleagris) is associated with a deletion of Phenylalanine-256 in the MC1R gene
    O Vidal
    Departament de Biologia, Universitat de Girona, Girona, Catalonia, Spain
    Anim Genet 41:656-8. 2010
    ..Interestingly, this is the first case of recessive melanism linked to MC1R...
  86. ncbi Chronic immune stimulation correlates with reduced phenylalanine turnover
    G Neurauter
    Division of Biological Chemistry, Biocenter, Innsbruck Medical University, Innsbruck, Austria
    Curr Drug Metab 9:622-7. 2008
    ..Recent data suggest that oxidative loss of BH4 in chronic inflammatory conditions can reduce the biosynthesis of catecholamines, which may relate to disturbed adrenergic neurotransmitter pathways in patients...
  87. pmc Structural basis for the entrance into the phenylpropanoid metabolism catalyzed by phenylalanine ammonia-lyase
    Holger Ritter
    Institut fur Organische Chemie und Biochemie, Albert Ludwigs Universitat, Freiburg im Breisgau, Germany 79104
    Plant Cell 16:3426-36. 2004
    ..A pathway of the catalyzed reaction is proposed in agreement with established biochemical data. The inactivation of the enzyme by a nucleophile is described in detail...
  88. pmc Purification and use of glycomacropeptide for nutritional management of phenylketonuria
    Caitlin E Laclair
    Dept of Food Science, Univ of Wisconsin, Madison, WI 53706, USA
    J Food Sci 74:E199-206. 2009
    Individuals with phenylketonuria (PKU) cannot metabolize phenylalanine (Phe) and must adhere to a low-Phe diet in which most dietary protein is provided by a Phe-free amino acid formula...
  89. ncbi Phenylalanine 171 is a molecular brake for translesion synthesis across benzo[a]pyrene-guanine adducts by human DNA polymerase kappa
    Akira Sassa
    Division of Genetics and Mutagenesis, National Institute of Health Sciences, 1 18 1 Kamiyoga, Setagaya Ku, Tokyo 158 8501, Japan
    Mutat Res 718:10-7. 2011
    ..Remarkably, the substitution of alanine for phenylalanine 171 (F171), an amino acid conserved between Pol κ and its bacterial counterpart Escherichia coli DinB, ..
  90. pmc Improved nutritional management of phenylketonuria by using a diet containing glycomacropeptide compared with amino acids
    Sandra C van Calcar
    Waisman Center, University of Wisconsin, Madison, WI 53706, USA
    Am J Clin Nutr 89:1068-77. 2009
    Phenylketonuria (PKU) requires a lifelong low-phenylalanine diet that provides the majority of protein from a phenylalanine-free amino acid (AA) formula...
  91. ncbi Disruption of a global regulatory gene to enhance central carbon flux into phenylalanine biosynthesis in Escherichia coli
    M Tatarko
    Department of Molecular Biology and Immunology, University of North Texas Health Science Center at Fort Worth, 3500 Camp Bowie Boulevard, Fort Worth, TX 76107 2699, USA
    Curr Microbiol 43:26-32. 2001
    ..A strain in which the aromatic (shikimate) pathway had been optimized produced twofold more phenylalanine when csrA was disrupted...
  92. ncbi Increased blood phenylalanine to tyrosine ratio in HIV-1 infection and correction following effective antiretroviral therapy
    Robert Zangerle
    HIV Unit, Department of Dermatology and Venerology, Innsbruck Medical University, Innsbruck, Austria
    Brain Behav Immun 24:403-8. 2010
    Higher blood levels of the essential amino acid phenylalanine (phe) have been documented in patients with HIV-1 infection. They may relate to a diminished conversion of phe to tyrosine (tyr) by the enzyme phenylalanine-hydroxylase (PAH)...
  93. pmc Regulation of phenylalanine hydroxylase: conformational changes upon phenylalanine binding detected by hydrogen/deuterium exchange and mass spectrometry
    Jun Li
    Department of Biochemistry and Biophysics, Texas A and M University, College Station, Texas 77843 2128, USA
    Biochemistry 49:3327-35. 2010
    b>Phenylalanine acts as an allosteric activator of the tetrahydropterin-dependent enzyme phenylalanine hydroxylase...
  94. ncbi PRESERVE-beta: two-year efficacy and safety of initial combination therapy with nateglinide or glyburide plus metformin
    John Gerich
    General Clinical Research Center, University of Rochester, New York, USA
    Diabetes Care 28:2093-9. 2005
    ..To compare long-term efficacy and safety of initial combination therapy with nateglinide/metformin versus glyburide/metformin...
  95. ncbi Blood flow restriction during low-intensity resistance exercise increases S6K1 phosphorylation and muscle protein synthesis
    Satoshi Fujita
    Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA
    J Appl Physiol (1985) 103:903-10. 2007
    ..We conclude that the activation of the mTOR signaling pathway appears to be an important cellular mechanism that may help explain the enhanced muscle protein synthesis during REFR...
  96. ncbi Decameric GTP cyclohydrolase I forms complexes with two pentameric GTP cyclohydrolase I feedback regulatory proteins in the presence of phenylalanine or of a combination of tetrahydrobiopterin and GTP
    T Yoneyama
    Department of Surgery, University of Pittsburgh, Pittsburgh, Pennsylvania 15213, USA
    J Biol Chem 273:20102-8. 1998
    ..of GTP cyclohydrolase I is inhibited by (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin (BH4) and stimulated by phenylalanine through complex formation with GTP cyclohydrolase I feedback regulatory protein (GFRP)...
  97. pmc RNAi suppression of Arogenate Dehydratase1 reveals that phenylalanine is synthesized predominantly via the arogenate pathway in petunia petals
    Hiroshi Maeda
    Department of Horticulture and Landscape Architecture, Purdue University, West Lafayette, Indiana 47907, USA
    Plant Cell 22:832-49. 2010
    ..These results provide genetic evidence that Phe is synthesized predominantly via arogenate in petunia petals and uncover a novel posttranscriptional regulation of the shikimate pathway...
  98. ncbi Increased urinary excretion of a 3-(3-hydroxyphenyl)-3-hydroxypropionic acid (HPHPA), an abnormal phenylalanine metabolite of Clostridia spp. in the gastrointestinal tract, in urine samples from patients with autism and schizophrenia
    William Shaw
    The Great Plains Laboratory, Inc, Lenexa, Kansas 66214, USA
    Nutr Neurosci 13:135-43. 2010
  99. ncbi Influence of peptide supply and cosubstrates on phenylalanine metabolism of Lactobacillus sanfranciscensis DSM20451(T) and Lactobacillus plantarum TMW1.468
    Nicoline Vermeulen
    Technische Mikrobiologie, Technische Universitat Munchen, Weihenstephaner Steig 16, D 85350 Freising, Germany
    J Agric Food Chem 54:3832-9. 2006
    ..One of these metabolites is phenyllactic acid (PLA), which is a catabolite from phenylalanine. In this work, the influence of peptide supply and cosubstrates was determined on PLA formation from ..
  100. ncbi Peak bone mass in patients with phenylketonuria
    D Modan-Moses
    Pediatric Endocrinology Unit, The Edmond and Lily Safra Children s Hospital, Tel Hashomer, 52621, Israel
    J Inherit Metab Dis 30:202-8. 2007
    ..Our aim was to evaluate peak bone mass in adult PKU patients and to relate BMD to nutritional parameters...
  101. ncbi Evaluation of nutritional status and pathophysiology of growth retardation in patients with phenylketonuria
    D Dobbelaere
    Unit of Metabolic Disease, Department of Pediatrics, Lille University Children s Hospital and Faculty of Medicine, Lille, France
    J Inherit Metab Dis 26:1-11. 2003 com-mon in people with phenylketonuria (PKU) during the first years of life while they receive a low-phenylalanine (Phe) diet...

Research Grants66

  1. Anthrax Toxins Impair Phagocyte Actin-based Motility
    Frederick Southwick; Fiscal Year: 2010
    ..A. Compare the effects of LT on formyl-methionly-leucyl-phenylalanine (FMLP), platelet activating factor (PAF) and IgG-immune complex induction of neutrophil actin assembly using ..
  2. Novel therapy for monoamine neurotransmitter deficiency in PKU
    Cary O Harding; Fiscal Year: 2013
    ..content of L-tyrosine and L-tryptophan, the substrates for dopamine and serotonin synthesis respectively, and phenylalanine- mediated competitive inhibition of tyrosine hydroxylase (TH) and tryptophan hydroxylase (TPH), the rate- ..
  3. TSQ Vantage Bundle with Acquity UltraPerformance LC
    Nicolaas E Deutz; Fiscal Year: 2010
    ..pathway substrates as metrics of intestinal health and activity;and (ii) stable (heavy) isotopomers of L-phenylalanine, 3-methyl-L-histidine, and other select macronutrients as metrics of protein synthesis and turnover. Since Drs...
  4. Cholesterol and Sphingolipid Perturbation of Membrane Traffic in Human Disease
    Andrew H Limper; Fiscal Year: 2011
    ..Some missense mutations, including deletion of phenylalanine 508 (?F508), the most common mutation in CF disease, cause retention of the protein in the ER and premature ..
  5. Augmented Phenylalanine Clearance by Muscles as Novel Therapy for Phenylketonuria
    Cary O Harding; Fiscal Year: 2013
    ..More specifically, the investigators will focus their efforts upon phenylalanine hydroxylase (PAH)-deficient Pahenu2 mice, a model of human phenylketonuria (PKU), and will evaluate expression ..
  6. Regulated biosynthesis and function of ABC-transport systems
    Patrick H Thibodeau; Fiscal Year: 2013
    ..The most common mutation, deletion of phenylalanine 508 (?F508) is associated with >90% of all CF patients...
  7. Development of AAV vectors for the phenotypic correction of sickle cell disease
    Angela Rivers; Fiscal Year: 2013
    ..Novel AAV capsids will be generated by two methods: (1) systematic mutation of tyrosine to phenylalanine and (2) DNA shuffling, which involves randomization of capsid amino acids of different AAV serotypes...
  8. Development of a Humanized Pig Model of Cystic Fibrosis
    Christopher Rogers; Fiscal Year: 2010
    ..Most CF patients have a mutation that deletes phenylalanine 508 (?F508) and prevents CFTR from being properly processed and delivered to cell membranes...
  9. Hyperphenylalaninemia in cerebral malaria
    DONALD LEE GRANGER; Fiscal Year: 2010
    ..that Tanzanian children with CM have markedly low plasma levels of arginine (Arg) and markedly elevated phenylalanine (Phe). Arg is needed for synthesis of nitric oxide (NO) by NO synthases (NOS)...
  10. Engineering a Supplemental Phenylalanine Metabolic Pathway to Prevent mPKU
    Stephen K Hunter; Fiscal Year: 2011
    The Hunter lab proposes to develop genetically modified cells to overproduce Phenylalanine Hydroxylase (PAH). This enzyme is frequently mutated and completely or partially inactive in persons with phenylketonuria (PKU)...
  11. Characterizing RNA-metal binding by Raman spectroscopy
    Paul R Carey; Fiscal Year: 2012
    ..RNAs, the Hepatitis Delta Virus (HDV) ribozyme, the P4-P6 domain and Twort ribozyme from group I introns, and phenylalanine tRNA...
  12. Mechanisms of FGFR2 Signaling in Salivary Gland Branching Morphogenesis
    Veraragavan P Eswarakumar; Fiscal Year: 2013
    ..In the first mutant mouse model, the four Grb2 binding sites are mutated to phenylalanine (4F), and thus cannot recruit Grb2...
  13. Phase 2 Study of Glycomacropeptide vs. Amino Acid Diet for the Management of PKU
    Denise M Ney; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Individuals with PKU lack the enzyme phenylalanine hydroxylase that is needed to metabolize the essential amino acid phenylalanine (phe)...
  14. Novel Pattern Specific Isotopic Labeling of Aromatic Amino Acids
    Robert McFeeters; Fiscal Year: 2010
    ..Specific Aromatic Labeling since isotope incorporation is at specific, known positions in the side chains of phenylalanine, tyrosine, and tryptophan...
    John R Riordan; Fiscal Year: 2013
    ..One of these domain-swapping interactions is mediated by the aromatic side chain of phenylalanine residue 508, deleted in most CF patients, which we showed independently is directly involved in channel gating...
  16. CFTR degradation in the early biogenesis
    Fei Sun; Fiscal Year: 2013
    ..A critical issue in CF disease is the inability of the most common CFTR mutation, deletion of the phenylalanine residue at position 508 (?F508CFTR), to achieve the native, folded state required for its export from the ..
  17. Role of LMTK2 in CFTR Trafficking
    Neil A Bradbury; Fiscal Year: 2013
    ..The most common mutation in CF is caused by the deletion of a phenylalanine residue at position 508 ([unreadable]F508) in CFTR...
  18. Correction of defective deltaF508-CFTR processing in cystic fibrosis
    Gergely L Lukacs; Fiscal Year: 2013
    ..The most prevalent CF mutation, deletion of phenylalanine 508 (?F508) disrupts the posttranslational folding, as well as the biosynthetic and endocytic processing of ..
    Peter W Schiller; Fiscal Year: 2013
    ..In Specific Aim 1, [unreadable] opioid peptide agonists containing novel phenylalanine analogues in place of tyrosine-1 will be synthesized...
  20. Tie Tek Modulation of Cardiac Development
    H Scott Baldwin; Fiscal Year: 2013 well as point mutations in critical tyrosine kinase domains in the cytoplasmic region of Tie1 (tyrosine to phenylalanine/Y1113F and lysine to alanine/K866A)...
  21. Cell Biology of CFTR in Polarized Epithelia
    James F Collawn; Fiscal Year: 2013
    ..The most common form of CF is caused by deletion of phenylalanine at position 508 ([unreadable]F508)...
  22. Vitreal delivery of novel AAV vectors for CNGA3 achromatopsia cone gene therapy
    Jijing Pang; Fiscal Year: 2013
    ..we will use a mouse model of human achromatopsia with CNGA3 mutations using several novel tyrosine to phenylalanine plus threonine to valine mutant capsid AAV vectors as the gene delivery vehicles for intravitreal injection...
    K Sreekumaran Nair; Fiscal Year: 2013
    ..Following in vivo metabolic labeling of proteins with a stable isotope of phenylalanine, different gel spot isoforms of an individual protein have widely variable isotopic enrichment...
  24. Modeling proteostatic deficiencies in CFTR
    Beverly H Koller; Fiscal Year: 2012
    ..of CF patients carry at least one CFTR allele with a three base pair deletion that results in the absence of a phenylalanine (F) in the first nucleotide binding domain of the CFTR protein...
    ROBERT JOHN COLLIER; Fiscal Year: 2012
    ..We identified a crucial structural feature of the pore, dubbed the Phe clamp and formed from the seven Phenylalanine 427 residues, and showed that the pH gradient across the membrane is the primary energy source for ..
    William R Skach; Fiscal Year: 2012
    ..that the primary defect in up to 90% of the 30,000 CF patients in the US is caused by deletion of a single phenylalanine residue at position 508...
  27. Improved Therapies for AML in the Elderly
    SARAH C NABINGER; Fiscal Year: 2011
    ..To examine this hypothesis we will Aim 1: (A) modify N51-Flt3 Y599 to phenylalanine (Y599F) in the first, the second, or both Y599 sites and utilize Baf3 cells to create stable cell lines;and (B)..
  28. Mothers' Experience of Breastfeeding Infants with Phenylketonuria (PKU)
    SANDRA ANN BANTA WRIGHT; Fiscal Year: 2010
    ..experiences of mothers'of infants with PKU who are breastfeeding and are required to supplement with phenylalanine-free formula...
  29. Protein and Peptide Derived Cofactors
    Judith P Klinman; Fiscal Year: 2013
    ..These studies use an unnatural amino acid, 4-amino-phenylalanine (p-AF) that has been successfully inserted at three key positions within the enzyme active site...
  30. Trans-NIH Research Support
    Raymond A Frizzell; Fiscal Year: 2013
    ..The most common CFTR mutation, present on at least one allele in >90% of CF patients, deletes phenylalanine at position 508 (F508del), which causes the protein to misfold...
  31. Roles of protein structure and diiron cluster chemistry in oxygen activation
    John D Lipscomb; Fiscal Year: 2013
    ..O2 only when it is complexed with its NRPS (CmlP) covalently loaded with the chloramphenicol precursor L-p-NH2-phenylalanine (PAPA) on its thiolation domain...
  32. Novel Chemotherapeutics for Brain Tumors
    Bernd Jandeleit; Fiscal Year: 2013
    ..The specificity for LAT1 vs. other amino acid transporters will be tested by [3H]-phenylalanine uptake competition in cells expressing those transporters...
  33. Cone opsins in photoreceptor degeneration
    Yingbin Fu; Fiscal Year: 2013
    ..We identified a phenylalanine-rich region in the short-wavelength opsin family (SW1) but absent from the medium/long-wavelength opsin family...
  34. MUC1 regulation of TGF-beta function in pancreatic cancer cells
    Pinku Mukherjee; Fiscal Year: 2013
    ..EMT in MUC1+, MUC1-, and MUC1+ cells in which the tyrosine residues in the MUC1 CT have been mutated to phenylalanine (MUC1 Y0);2) to determine the differential effects of TGF-? induced apoptosis in MUC1+, MUC1-, and MUC1 Y0 ..
  35. A Novel HTS Cell-Based Imaging Assay To Identify Chemical Correctors for ??F508-C
    Wook Joon Chung; Fiscal Year: 2009
    ..The most prevalent mutation is a deletion of phenylalanine at position 508 (?F508-CFTR) that causes defects in folding and trafficking of CFTR and a loss of apical ..
  36. Molecular Mechanisms Controlling Endocytic Recycling
    STEVEN H CAPLAN; Fiscal Year: 2013
    ..EH-domains interact with proteins containing the tripeptide motif asparagine-proline- phenylalanine (NPF)...
    UTTAM RAJBHANDARY; Fiscal Year: 2009
    ..These include (1) crosslinking of IF2 carrying benzoyl-phenylalanine residues at specific sites to fMet-tRNA and identification of crosslinking sites, (2) isolation of suppressor ..
  38. Halogenated Insulin: a fast-acting, ultra stable analog.
    BRUCE HILL FRANK; Fiscal Year: 2010 component of HumalogTM) containing a single chloro-substitution within the aromatic ring of a conserved phenylalanine in the B-chain (PheB24). Insulin-Cl retains the interchange of residues B28 and B29 (substitutions ProB28 ...
  39. Ocular Disposition of Antimicrobial Agents
    Ashim K Mitra; Fiscal Year: 2010
    ..application are a) To synthesize derivatives of erythromycin, levofloxacin and ofloxacin targeting amino acid (phenylalanine and glutamate prodrugs), peptide (valine, valine-valine and glycine-valine prodrugs) and ascorbate (ascorbic ..
  40. Three-Dimensional Super-Resolution Microscopy Study of Nuclear Transport
    Weidong Yang; Fiscal Year: 2013
    ..Translocation of larger molecules (up to 50 MDa) is hindered by the phenylalanine- glycine (FG) nucleoporin (Nups) barrier inside the NPCs unless they are chaperoned by transport receptors...
  41. Single-Molecule Three-Dimensional Snapshots of Nuclear Transport
    Weidong Yang; Fiscal Year: 2010
    ..However, translocation of large molecules (up to 50 MDa) is hindered by the phenylalanine-glycine (FG) repeats barrier inside the NPCs unless they are chaperoned by transport receptors...
  42. Direct visualization of the FG nups, the selective gating for nuclear transport
    Loren E Hough; Fiscal Year: 2010 lined with proteins rich in hydrophilic natively disordered regions containing degenerate repeated phenylalanine-glycine (FG) motifs termed "FG nups"...
  43. Regulation of Phenylalanine Catabolism
    Paul F Fitzpatrick; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): The liver enzyme phenylalanine hydroxylase is responsible for catabolism of excess phenylalanine in the diet...
  44. Architecture of the Diffusion Barrier in the Nuclear Pore Complex
    Michael F Rexach; Fiscal Year: 2010
    ..nups) that feature large unfolded domains in their native functional state, which are decorated with multiple phenylalanine glycine motifs (FG domains)...
    George Dunaway; Fiscal Year: 1991
    ..To this end, we have successfully developed a method for measuring rates of incorporation of radioactive phenylalanine into the different types of subunits...
  46. Battling AIDS via Mechanistic Understanding of the tRNA Phe modification enzyme T
    Lei Li; Fiscal Year: 2013
    ..goal of this research is to reveal the missing substrate "X" and shed light on the reaction mechanism for a phenylalanine transfer RNA (tRNAPhe) modification enzyme TYW1...
    RICHARD MOXLEY; Fiscal Year: 1992
    ..of insulin in the low, middle and high physiologic ranges, infusion of the stable isotope, L-(ring 2H5)-phenylalanine during the glucose clamp procedures will allow measurement of the effectiveness of insulin in controlling the ..
  48. Aging, AMP Kinase and Skeletal Muscle Overload
    Scott Gordon; Fiscal Year: 2005
    ..PLT) muscles, AMPK phosphorylation/activity, rates of mixed and myofibrillar protein synthesis (via 3H- phenylalanine incorporation), AMPK-y subunits influencing AMPK activity, and potential signaling intermediates controlling ..
    KENNETH KARLIN; Fiscal Year: 2002
    ..We plan to study: (1) Cu-pterin interactions, to help elucidate the active site chemistry of Cu-phenylalanine hydroxylase. Chelating pterins will be used to study Cu/O2 reactions with reduced pterins and substrates...
  50. Effects of Formyl Peptide Receptor variants on function
    John Mills; Fiscal Year: 2005
    ..that their neutrophils demonstrated poor chemotactic response toward the formyl peptide, methionine-leucine-phenylalanine (fMLP)...
    RAYMOND STEVENS; Fiscal Year: 2004
    The three-dimensional structure of the enzymes phenylalanine hydroxylase and tryptophan hydroxylase will be studied using crystallographic methods...
    PETER FARRELL; Fiscal Year: 1999
    ..Rates of protein synthesis will be measured by incorporation of 3H-Phenylalanine using either an in vivo tritiated phenylalanine flooding dose technique or in situ bilateral hindlimb ..
  53. Mechanisms of Neurotransmitter Biosynthesis
    Paul F Fitzpatrick; Fiscal Year: 2011
    b>Phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase make up the family of non-heme iron pterin-dependent aromatic amino acid hydroxylases...
    Mimi Takami; Fiscal Year: 2003
    ..of well characterized receptors such as the chemokine receptors, IL-8, C5a, and N-Formylmethionlleucyl- phenylalanine (FMLP), cytokine receptors, such as TNF, and activation of Protein Kinase C...
  55. Rescue of CFTR-deltaF508 misfolding by pharmacological and genetic suppressors
    RYAN TYLER; Fiscal Year: 2009 nearly 90% of cystic fibrosis patients in North America contains a trinucleotide deletion that removes the phenylalanine at position 508 of the CFTR peptide sequence...
  56. Mechanisms of catalysis by an alpha-amino acid dehydrogenase
    Harvey F Fisher; Fiscal Year: 2011
    ..reaction step surface. As a hypothesis we offer a newly extended mechanism for the L-phenylalanine dehydrogenase reaction which includes both steps and complexes whose occurrence is well established and ..
    Brian Edwards; Fiscal Year: 1993
    ..or substrate specificity, with the intention of delineating further the roles played by aspartic acid-121 and phenylalanine-120 in establishing the catalytic power and substrate specificity of this enzyme...
  58. Genetic Reconstitution for Phenylketonuria
    Savio Woo; Fiscal Year: 2006
    ..PKU) is a recessive monogenic disorder in amino acid metabolism that results from a deficiency of hepatic phenylalanine hydroxylase (PAH), and it predisposes affected individuals to severe and permanent mental retardation...
    Kenzo Sato; Fiscal Year: 2000
    ..The most common mutation is the loss of a phenylalanine at position 508 (deltaF508). deltaF508 comprises approximately 70% of CF patients...
    MALONNE DAVIES; Fiscal Year: 2002
    ..b>Phenylalanine will be determined in plasma in a similar manner using a copper electrode...
    JUNE AYLING; Fiscal Year: 1990
    The three aromatic amino acid hydroxylases are responsible for the metabolism of excess ditary phenylalanine, and initiating the biosynthesis of epinephrine and the catecholamine and serotonin neurotransmitters...
  62. Hyperphenylalaninemia in cerebral malaria
    DONALD GRANGER; Fiscal Year: 2006
    ..that Tanzanian children with CM have markedly low plasma levels of arginine (Arg) and markedly elevated phenylalanine (Phe). Arg is needed for synthesis of nitric oxide (NO) by NO synthases (NOS)...
    RANDY EISENSMITH; Fiscal Year: 1999
    ..combination if necessary, and if synergism is likely based on immunological studies, by expressing the mouse phenylalanine hydroxylase (PAH) gene in PAH-deficient Pahenu2 mice, an animal model of phenylketonuria...
    John Caradonna; Fiscal Year: 2003 to elucidate the chemical and physical properties of the mononuclear non-heme iron dependent metalloenzyme, phenylalanine hydroxylase (PAH, phenylalanine 4-monooxygenase, E.C.
    EDGAR RILEY; Fiscal Year: 1980
    Our objectives are (1) to compare tumor induction by X-irradiation, by simulated chemotherapeutic regimens of L-phenylalanine mustard (L-PAM) or 5-Fluorouracil (5-FU) and by combined modalities and (2) to learn more about the properties ..
    Roy Jensen; Fiscal Year: 1992
    Man lacks a biochemical pathway for synthesis of aromatic amino acids. Thus, L-tyrosine, L-phenylalanine, L-tryptophan and vitamin-like derivatives of the pathway must be supplied externally...