phenylalanine

Summary

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

Top Publications

  1. 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
  2. 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
  3. doi 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. doi 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
  9. 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
  10. 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

Research Grants

Detail Information

Publications305 found, 100 shown here

  1. 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 ..
  2. 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)...
  3. doi 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...
  4. 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)...
  5. 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...
  6. 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...
  7. 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 ..
  8. doi 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
    ....
  9. 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 ..
  10. 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...
  11. doi 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)...
  12. 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)...
  13. 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...
  14. 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...
  15. 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...
  16. 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 ..
  17. 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...
  18. 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...
  19. 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...
  20. 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...
  21. 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...
  22. 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 ..
  23. 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...
  24. 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...
  25. 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
    ....
  26. 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
    ....
  27. 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 ..
  28. 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...
  29. doi 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...
  30. 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...
  31. 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...
  32. 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
    ..is 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 ..
  33. 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...
  34. doi 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...
  35. 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...
  36. 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)...
  37. doi 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, ..
  38. 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
    ....
  39. 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...
  40. 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...
  41. 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...
  42. 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 ..
  43. 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...
  44. doi 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...
  45. 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...
  46. 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)...
  47. doi 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)...
  48. 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)...
  49. 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)...
  50. ncbi Crystal structure of phenylalanine ammonia lyase: multiple helix dipoles implicated in catalysis
    Joseph C Calabrese
    DuPont Central Research and Development, Experimental Station, Wilmington, Delaware 19880 0228, USA
    Biochemistry 43:11403-16. 2004
    The first three-dimensional structure of phenylalanine ammonia lyase (PAL) has been determined at 2.1 A resolution for PAL from Rhodosporidium toruloides...
  51. ncbi Use of phenylalanine-to-tyrosine ratio determined by tandem mass spectrometry to improve newborn screening for phenylketonuria of early discharge specimens collected in the first 24 hours
    D H Chace
    1 Neo Gen Screening, 110 Roessler Road, Pittsburgh, PA 15220, USA
    Clin Chem 44:2405-9. 1998
    ..In addition to the detection of PKU, MS/MS can also detect other aminoacidopathies and disorders of fatty acid and organic acid metabolism with lower false-positive rates than other methods currently used in newborn screening programs...
  52. 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...
  53. pmc Dopamine precursor depletion improves punishment prediction during reversal learning in healthy females but not males
    Oliver J Robinson
    Department of Psychiatry and Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, Addenbrooke s Hospital, P O Box 189, Level E4, Hills Road, Cambridge, CB2 2QQ, UK
    Psychopharmacology (Berl) 211:187-95. 2010
    ..Here, we extend these prior findings by examining the effects of artificially reducing DA synthesis in healthy individuals performing this previously employed task...
  54. 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...
  55. doi 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
    ....
  56. 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
    ..is com-mon in people with phenylketonuria (PKU) during the first years of life while they receive a low-phenylalanine (Phe) diet...
  57. 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 ..
  58. doi Management of phenylketonuria in Europe: survey results from 19 countries
    Nenad Blau
    University Children s Hospital, Zurich, Switzerland
    Mol Genet Metab 99:109-15. 2010
    ..The greatest variability existed especially in the definition of PKU phenotypes, therapeutic blood phenylalanine (Phe) target concentrations, and follow-up practices for PKU patients...
  59. doi Acute dopamine and/or serotonin depletion does not modulate mismatch negativity (MMN) in healthy human participants
    Sumie Leung
    Brain Sciences Institute, Faculty of Life and Social Sciences, Swinburne University of Technology, P O Box 218, John Street Hawthorn, 3122, Melbourne, Vic, Australia
    Psychopharmacology (Berl) 208:233-44. 2010
    ..While the neurochemical basis of MMN has been linked to N-methyl-D: -aspartic acid (NMDA) receptor function, the roles of the dopaminergic and/or the serotonergic systems are not fully explored in humans...
  60. ncbi Repair by Src kinase of function-impaired RET with multiple endocrine neoplasia type 2A mutation with substitutions of tyrosines in the COOH-terminal kinase domain for phenylalanine
    Masashi Kato
    Department of Immunology, Nagoya University Graduate School of Medicine, 65 Tsurumai Cho, Showa Ku, Nagoya, Aichi 466 8550, Japan
    Cancer Res 62:2414-22. 2002
    ..In this study, we compared the activities of mutant RET-MEN2A with substitutions of each of nine tyrosines for phenylalanine (Y1062F, Y1015F, Y981F, Y952F, Y928F, Y905F, Y900F, Y864F, and Y826F), which had been transfected into NIH 3T3 ..
  61. doi Experimental evidence that phenylalanine provokes oxidative stress in hippocampus and cerebral cortex of developing rats
    Carolina G Fernandes
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal de Rio Grande do Sul, Rua Ramiro Barcelos No 2600, Anexo, Porto Alegre, RS, CEP 90035 003, Brazil
    Cell Mol Neurobiol 30:317-26. 2010
    High levels of phenylalanine (Phe) are the biochemical hallmark of phenylketonuria (PKU), a neurometabolic disorder clinically characterized by severe mental retardation and other brain abnormalities, including cortical atrophy and ..
  62. doi Introduction of a stress-responsive gene, yggG, enhances the yield of L-phenylalanine with decreased acetic acid production in a recombinant Escherichia coli
    Yoshihiro Ojima
    Division of Chemical Engineering, Department of Materials Engineering Science, Graduate School of Engineering Science, Osaka University, Toyonaka, Osaka 560 8531, Japan
    Biotechnol Lett 31:525-30. 2009
    A stress-responsive gene, yggG, was introduced into an L-phenylalanine producer, Escherichia coli AJ12741. In shake-flask culture, the yggG-containing recombinant strain (named AJ12741/pHYGG) produced 6...
  63. ncbi Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria
    S C J Huijbregts
    Department of Clinical Neuropsychology, Vrije Universiteit Amsterdam, The Netherlands
    Neuropsychology 17:369-79. 2003
    ..Correlations with historical phenylalanine levels indicated a later maturation of the level of control required by the pursuit task compared with the ..
  64. doi Neuropsychological speed tests and blood phenylalanine levels in patients with phenylketonuria: a meta-analysis
    Julia Albrecht
    Centre for Paediatric and Adolescent Medicine, University of Heidelberg, Im Neuenheimer Feld 430, D 69120 Heidelberg, Germany
    Neurosci Biobehav Rev 33:414-21. 2009
    Although pathogenesis of phenylketonuria is not completely understood, a low phenylalanine diet is effective to prevent severe neurological impairment, mental retardation and behavioural difficulties...
  65. ncbi Relationship between myelin production and dopamine synthesis in the PKU mouse brain
    Bernadin Joseph
    Department of Neurology, The University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Neurochem 86:615-26. 2003
    Phenylketonuria is caused by specific mutations in the phenylalanine hydroxylase gene and is characterized by elevated blood phenylalanine levels, hypomyelination in forebrain structures, reduced dopamine levels, and cognitive ..
  66. ncbi Immunoadsorption plasmapheresis using a phenylalanine column as an effective treatment for lupus nephritis
    Kaoru Sugimoto
    Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan
    Ther Apher Dial 10:187-92. 2006
    ..The purpose of this study was to assess patients with lupus nephritis (LN) treated by IAPP using a phenylalanine column and determine its efficacy with respect to conventional therapies...
  67. ncbi Importance of entropy in the conformational equilibrium of phenylalanine: a matrix-isolation infrared spectroscopy and density functional theory study
    A Kaczor
    Department of Chemistry, University of Coimbra, P 3004 535 Coimbra, Portugal
    J Phys Chem A 110:2360-70. 2006
    The conformational behavior and infrared spectrum of l-phenylalanine were studied by matrix-isolation infrared spectroscopy and DFT [B3LYP/6-311++G(d,p)] calculations...
  68. ncbi Membrane type 4 matrix metalloproteinase (MT4-MMP, MMP-17) is a glycosylphosphatidylinositol-anchored proteinase
    Y Itoh
    Department of Cancer Cell Research, Institute of Medical Science, University of Tokyo, 4 6 1 Shirokanedai, Minato ku, Tokyo 108 8639, Japan
    J Biol Chem 274:34260-6. 1999
    ..GPI anchoring of MT4-MMP on the cell surface indicates a unique biological function and character for this proteinase...
  69. ncbi Phenylalanine blood levels and clinical outcomes in phenylketonuria: a systematic literature review and meta-analysis
    Susan E Waisbren
    Children s Hospital Boston, 1 Autumn Street, Room 525, Boston, MA 02115, USA
    Mol Genet Metab 92:63-70. 2007
    Blood phenylalanine (Phe) levels provide a practical and reliable method for the diagnosis and monitoring of metabolic status in patients with phenylketonuria (PKU)...
  70. pmc Characterization of the Pseudomonas aeruginosa transcriptional response to phenylalanine and tyrosine
    Gregory C Palmer
    Section of Molecular Genetics and Microbiology, Institute of Cell and Molecular Biology, The University of Texas at Austin, 1 University Station, A5000, Austin, TX 78712, USA
    J Bacteriol 192:2722-8. 2010
    ..Our work characterizes a transcriptional regulator of catabolic genes induced during P. aeruginosa growth in CF sputum...
  71. ncbi The site-specific incorporation of p-iodo-L-phenylalanine into proteins for structure determination
    Jianming Xie
    Nat Biotechnol 22:1297-301. 2004
    ..We now show that this technology can be used to efficiently and site-specifically incorporate p-iodo-L-phenylalanine (iodoPhe) into proteins in response to an amber TAG codon...
  72. pmc Tyrosine and phenylalanine are synthesized within the plastids in Arabidopsis
    Pascal Rippert
    Institut National de la Recherche Agronomique, UMR 1200, F 38054 Grenoble, France
    Plant Physiol 149:1251-60. 2009
    ..now, the only known destiny of prephenate, the product of CM, is incorporation into tyrosine (Tyr) and/or phenylalanine (Phe)...
  73. ncbi Focal adhesion kinase-dependent apoptosis of melanoma induced by tyrosine and phenylalanine deficiency
    Y M Fu
    Department of Pharmaceutical Sciences, College of Pharmacy, and the Cancer Prevention and Research Center, Washington State University, Pullman 99164 6510, USA
    Cancer Res 59:758-65. 1999
    We found previously that restriction of tyrosine (Tyr) and phenylalanine (Phe) inhibited growth and metastasis of B16BL6 murine melanoma and arrested these cells in the G0-G1 phase of the cell cycle...
  74. ncbi Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997
    P Burgard
    Fachrichtung Psychologie, Universitat des Saarlandes, Im Stadtwald, Saarbrucken, Germany
    Eur J Pediatr 158:46-54. 1999
    Treatment of hyperphenylalaninaemias due to phenylalanine hydroxylase deficiency with a low phenylalanine (Phe) diet is highly successful in preventing neurological impairment and mental retardation...
  75. ncbi A new strategy for the site-specific modification of proteins in vivo
    Zhiwen Zhang
    Department of Chemistry and Skaggs Institute for Chemical Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, California 92037, USA
    Biochemistry 42:6735-46. 2003
    ..the selection of an orthogonal tRNA-TyrRS pair that selectively and efficiently incorporates m-acetyl-l-phenylalanine into proteins in E. coli...
  76. ncbi The aromatic binding site for tetraethylammonium ion on potassium channels
    L Heginbotham
    Department of Neurobiology, Harvard Medical School, Boston, Massachusetts 02115
    Neuron 8:483-91. 1992
    ..A Shaker K+ channel can be made highly sensitive by introducing a tyrosine (or phenylalanine) at residue 449 in each of the four subunits...
  77. ncbi Placental transport of leucine, phenylalanine, glycine, and proline in intrauterine growth-restricted pregnancies
    C L Paolini
    Department of Obstetrics and Gynecology, University of Milan, 20142 Milan, Italy
    J Clin Endocrinol Metab 86:5427-32. 2001
    L-[1-13C]Leucine, [1-13C]glycine, L-[1-13C]phenylalanine, and L-[1-13C]proline were infused as a bolus into the maternal circulation of seven appropriate for gestational age at 30.3 +/- 3...
  78. ncbi Binding of peptides with basic and aromatic residues to bilayer membranes: phenylalanine in the myristoylated alanine-rich C kinase substrate effector domain penetrates into the hydrophobic core of the bilayer
    Wenyi Zhang
    Department of Biochemistry and Cell Biology, Center for Structural Biology, State University of New York, Stony Brook, NY 11794 5115, USA
    J Biol Chem 278:21459-66. 2003
    ....
  79. pmc Repression of aromatic amino acid biosynthesis in Escherichia coli K-12
    K D Brown
    J Bacteriol 108:386-99. 1971
    ..In the wild type, DAHP synthetase (phe) was multivalently repressed by phenylalanine plus tryptophan, whereas DAHP synthetase (tyr), chorismate mutase T-prephenate dehydrogenase, and transaminase ..
  80. ncbi Co-ingestion of protein and leucine stimulates muscle protein synthesis rates to the same extent in young and elderly lean men
    Rene Koopman
    Department of Human Biology, Maastricht University, Maastricht, Netherlands
    Am J Clin Nutr 84:623-32. 2006
    ..The progressive loss of skeletal muscle mass with aging is attributed to a disruption in the regulation of skeletal muscle protein turnover...
  81. ncbi The region from phenylalanine-17 to phenylalanine-28 of a yeast mitochondrial ATPase inhibitor is essential for its ATPase inhibitory activity
    N Ichikawa
    Department of Food and Nutrition, Faculty of Human Life Science, Osaka City University, Sugimoto, Sumiyoshi, Osaka 558 8585, Japan
    J Biochem 130:687-93. 2001
    ..These results show that the region from the position 17 to 28 of the yeast inhibitor is the most important for its activity and is required for the inhibition of F(1), rather than binding to the enzyme...
  82. pmc Phenylalanine-427 of anthrax protective antigen functions in both pore formation and protein translocation
    Jianjun Sun
    Department of Microbiology and Molecular Genetics, Harvard Medical School, 200 Longwood Avenue, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 105:4346-51. 2008
    ..b>Phenylalanine-427 (F427), a solvent-exposed residue in the lumen of the pore, was identified earlier as being crucial for ..
  83. ncbi Evaluation of apoptosis and micronucleation induced by reactor neutron beams with two different cadmium ratios in total and quiescent cell populations within solid tumors
    S Masunaga
    Radiation Oncology Research Laboratory, Research Reactor Institute, Kyoto University, Sennan gun, Osaka, Japan
    Int J Radiat Oncol Biol Phys 51:828-39. 2001
    ....
  84. pmc Molecular dissection of a quantitative trait locus: a phenylalanine-to-tyrosine substitution in the transmembrane domain of the bovine growth hormone receptor is associated with a major effect on milk yield and composition
    Sarah Blott
    Department of Genetics, Faculty of Veterinary Medicine, University of Liege, 4000 Liege, Belgium
    Genetics 163:253-66. 2003
    ....
  85. pmc FG/FxFG as well as GLFG repeats form a selective permeability barrier with self-healing properties
    Steffen Frey
    Max Planck Institut fur biophysikalische Chemie, Am Fassberg, Gottingen, Germany
    EMBO J 28:2554-67. 2009
    ..NTRs not only left the barrier intact, they even tightened it against passive influx, pointing to a role for NTRs in establishing and maintaining the permeability barrier of NPCs...
  86. doi Site-specific incorporation of p-propargyloxyphenylalanine in a cell-free environment for direct protein-protein click conjugation
    Bradley C Bundy
    Department of Chemical Engineering, Stanford University, Stanford, California 94305 5025, USA
    Bioconjug Chem 21:255-63. 2010
    ..Previously, incorporation of another CuAAC-compatible unnatural amino acid p-azido-l-phenylalanine (pAz) was demonstrated with CFPS...
  87. ncbi Ornithine decarboxylase promotes catalysis by binding the carboxylate in a buried pocket containing phenylalanine 397
    Laurie K Jackson
    Department of Pharmacology, The University of Texas Southwestern Medical Center at Dallas, 5323 Harry Hines Boulevard, Dallas, Texas 75390 9041, USA
    Biochemistry 42:2933-40. 2003
    ....
  88. doi 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...
  89. ncbi Double blind placebo control trial of large neutral amino acids in treatment of PKU: effect on blood phenylalanine
    R Matalon
    Department of Pediatrics, University of Texas Medical Branch, Children s Hospital, Galveston, Texas, USA
    J Inherit Metab Dis 30:153-8. 2007
    ..used on a limited number of patients with phenylketonuria (PKU) with the purpose of decreasing the influx of phenylalanine (Phe) to the brain...
  90. ncbi The effects of acute tyrosine and phenylalanine depletion on spatial working memory and planning in healthy volunteers are predicted by changes in striatal dopamine levels
    Mitul A Mehta
    PET Psychiatry, Cyclotron Building, MRC Clinical Sciences Centre, Imperial College, Hammersmith Hospital, London, W12 0NN, UK
    Psychopharmacology (Berl) 180:654-63. 2005
    ..However, the findings from studies in humans to date are mixed. While this may be due to the characteristics of the tasks used, it is also possible that these findings are explained by variable central effects of the manipulations used...
  91. ncbi Alpha4-integrin antagonism--an effective approach for the treatment of inflammatory diseases?
    Richard J Davenport
    Global Chemistry, UCB Group, Granta Park, Great Abington, Cambridge CB16GS, UK
    Drug Discov Today 12:569-76. 2007
    ..Recent advances in small molecule development are worth reviewing. New understanding of pharmacokinetics and selectivity will potentially contribute to the development of alpha4 antagonist with greater clinical efficacy and safety...
  92. doi Serum phenylalanine concentrations in patients with ovarian carcinoma correlate with concentrations of immune activation markers and of isoprostane-8
    Gabriele Neurauter
    Division of Biological Chemistry, Biocenter, Innsbruck Medical University, Fritz Pregl Strasse 3, 6020 Innsbruck, Austria
    Cancer Lett 272:141-7. 2008
    Increased blood concentrations of essential amino acid phenylalanine are common in patients with HIV infection, in trauma and sepsis and in patients with cancer. The reason for this phenomenon is still unclear...
  93. ncbi A randomized trial of long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria
    Carlo Agostoni
    Department of Pediatrics, University of Milan Medical School, Milan, Italy
    Dev Med Child Neurol 48:207-12. 2006
    ..double-blind, randomized study to investigate the effects on biochemical and physiological outcomes of a phenylalanine-free infant formula containing a fat blend supplemented with the long-chain polyunsaturated fatty acids (LC-..
  94. pmc The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating
    Liying Cui
    Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, 27599, USA
    J Physiol 572:347-58. 2006
    ..To explore the possible role of the phenylalanine aromatic side chain in channel gating we introduced a cysteine at this position in cysless CFTR, enabling its ..
  95. ncbi Analysis of the C-terminal structure of urinary Tamm-Horsfall protein reveals that the release of the glycosyl phosphatidylinositol-anchored counterpart from the kidney occurs by phenylalanine-specific proteolysis
    S Fukuoka
    Graduate School of Agriculture, Kyoto University, 611 0011 Uji, Kyoto, Japan
    Biochem Biophys Res Commun 289:1044-8. 2001
    ....
  96. doi Executive function in early-treated phenylketonuria: profile and underlying mechanisms
    Shawn E Christ
    Department of Psychological Sciences, 210 McAlester Hall, University of Missouri, Columbia, MO 65203, USA
    Mol Genet Metab 99:S22-32. 2010
    ..Cohort (e.g., age, phenylalanine (Phe) levels) and task (e.g...
  97. pmc Metabolism of primed, constant infusions of [1,2-¹³C₂] glycine and [1-¹³C₁] phenylalanine to urinary oxalate
    John Knight
    Department of Urology, Wake Forest University Medical Center, Winston Salem, NC 27157, USA
    Metabolism 60:950-6. 2011
    Experiments in humans and rodents using oral doses of glycine and phenylalanine have suggested that the metabolism of these amino acids contributes to urinary oxalate excretion...
  98. doi Blood phenylalanine control in phenylketonuria: a survey of 10 European centres
    K Ahring
    Department of PKU, Kennedy Centre, Glostrup, Denmark
    Eur J Clin Nutr 65:275-8. 2011
    Only limited data are available on the blood phenylalanine (Phe) concentrations achieved in European patients with phenylketonuria (PKU) on a low-Phe diet.
  99. pmc A phenylalanine clamp catalyzes protein translocation through the anthrax toxin pore
    Bryan A Krantz
    Department of Microbiology and Molecular Genetics, Harvard Medical School, 200 Longwood Avenue, Boston, MA 02115, USA
    Science 309:777-81. 2005
    ..We found that, during conversion of the heptameric precursor to the pore, the seven phenylalanine-427 residues converged within the lumen, generating a radially symmetric heptad of solvent-exposed aromatic ..
  100. ncbi Effects of tolbutamide and N-benzoyl-D-phenylalanine (NBDP) on the regulation of [Ca2+]i oscillations in mouse pancreatic islets
    S Lenzen
    Institute of Clinical Biochemistry, Hannover Medical School, D-30623, Hannover, Germany
    Biochem Pharmacol 62:923-8. 2001
    The sulfonylurea derivative, tolbutamide, and the phenylalanine derivative, N-benzoyl-D-phenylalanine (NBDP), both of which stimulate insulin secretion through interaction with the sulfonylurea receptor (SUR1), were studied for their ..
  101. pmc Preclinical evaluation of multiple species of PEGylated recombinant phenylalanine ammonia lyase for the treatment of phenylketonuria
    Christineh N Sarkissian
    Department of Biology, McGill University, Montreal, QC, Canada H3A 1B1
    Proc Natl Acad Sci U S A 105:20894-9. 2008
    Phenylketonuria (PKU) is a metabolic disorder, in which loss of phenylalanine hydroxylase activity results in neurotoxic levels of phenylalanine...

Research Grants84

  1. Oncogenic Kit receptor signaling in vivo
    Peter Besmer; Fiscal Year: 2010
    ..modified the Kit gene in the mouse genome by substituting critical tyrosine residues in the Kit protein with phenylalanine. These substitution mutations in the Kit receptor block either PI 3-kinase or SFK activation...
  2. Ocular Disposition of Antimicrobial Agents
    Ashim Mitra; Fiscal Year: 2007
    ..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 ..
  3. 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 ..
  4. Ocular Disposition of Antimicrobial Agents
    Ashim Mitra; Fiscal Year: 2009
    ..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 ..
  5. Aromatic amino acid metabolism in the pathogenesis of kwashiorkor
    Farook Jahoor; Fiscal Year: 2007
    ..We propose that decreased supply of aromatic amino acids (AAAs) is a factor. Because phenylalanine and tyrosine are precursors for dopamine, melanin and the catecholamines, and tryptophan is the precursor of ..
  6. Characterizing RNA-metal binding by Raman spectroscopy
    Paul R Carey; Fiscal Year: 2010
    ..RNAs, the Hepatitis Delta Virus (HDV) ribozyme, the P4-P6 domain and Twort ribozyme from group I introns, and phenylalanine tRNA...
  7. Characterizing RNA-metal binding by Raman spectroscopy
    Paul Carey; Fiscal Year: 2009
    ..RNAs, the Hepatitis Delta Virus (HDV) ribozyme, the P4-P6 domain and Twort ribozyme from group I introns, and phenylalanine tRNA...
  8. Functional Variants of the Human TPH2 Gene
    Marc Caron; Fiscal Year: 2009
    ..a high degree of sequence conservation between TPH1 and TPH2 and other mono-oxygenases for tyrosine (TH) and phenylalanine (PAH)...
  9. Functional Variants of the Human TPH2 Gene
    Marc Caron; Fiscal Year: 2009
    ..a high degree of sequence conservation between TPH1 and TPH2 and other mono-oxygenases for tyrosine (TH) and phenylalanine (PAH)...
  10. Mechanisms of FGFR2 Signaling in Salivary Gland Branching Morphogenesis
    Veraragavan P Eswarakumar; Fiscal Year: 2010
    ..In the first mutant mouse model, the four Grb2 binding sites are mutated to phenylalanine (4F), and thus cannot recruit Grb2...
  11. Amino Acids, Protein, and BMI in Adults Across Cultures
    Ka He; Fiscal Year: 2007
    ..arginine, aspartic acid, cystine, glutamic acid, glycine, histidine, isoleucine, leucine, lysine, methionine, phenylalanine, proline, serine, threonine, tryptophan, tyrosine, and valine, and a related compound - mono-sodium glutamate (..
  12. 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 ..
  13. Mechanisms of Aromatic Amino Acid Hydroxylases
    JORGE PAVON; Fiscal Year: 2007
    b>Phenylalanine hydroxylase (PheH), tyrosine hydroxylase (TyrH) and tryptophan hydroxylase (TrpH) make up the family of tetrahydropterin dependent hydroxylases...
  14. Metabolic Engineering as Therapy for Murine PKU
    Cary Harding; Fiscal Year: 2002
    ..adeno-associated virus (rAAV) vectors in the treatment of phenylketonuria (PKU) using, as a model, the phenylalanine hydroxylase (PAH) deficient Pahenu2 mouse...
  15. GASTRIC MUCOSAL INJURY ROLE OF INFLAMMATORY MEDIATORS
    LAURENCE CHEUNG; Fiscal Year: 1993
    ..the proposed, studies are designed to: 1) examine the effects of endotoxin, FMLP (formyl-leucyl-methionyl-phenylalanine, a chemotactic peptide released from bacteria), and tumor necrosis factor on the gastric microcirculation...
  16. 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)...
  17. Cell Biology of CFTR in Polarized Epithelia
    James F Collawn; Fiscal Year: 2010
    ..The most common form of CF is caused by deletion of phenylalanine at position 508 (?F508)...
  18. Cell Biology of CFTR in Polarized Epithelia
    James Collawn; Fiscal Year: 2009
    ..The most common form of CF is caused by deletion of phenylalanine at position 508 (?F508)...
  19. STRUCTURAL UNDERSTANDING OF PHENYLKETONURIA (PKU)
    RAYMOND STEVENS; Fiscal Year: 2004
    The three-dimensional structure of the enzymes phenylalanine hydroxylase and tryptophan hydroxylase will be studied using crystallographic methods...
  20. Cholesterol and Sphingolipid Perturbation of Membrane Traffic in Human Disease
    Richard Pagano; Fiscal Year: 2009
    ..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 ..
  21. SYNTHESIS AND BIOSYNTHESIS IN THE SHIKIMATE PATHWAY
    Bruce Ganem; Fiscal Year: 1999
    ..among these objectives is the study of chorismate mutase (CM), which catalyzes the first committed step in phenylalanine and tyrosine biosynthesis...
  22. 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...
  23. Mechanisms of Neurotransmitter Biosynthesis
    Paul Fitzpatrick; Fiscal Year: 2009
    b>Phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase make up the family of non-heme iron pterin-dependent aromatic amino acid hydroxylases...
  24. GENETIC ANALYSIS OF THE PLANT DEFENSE RESPONSE
    FREDERICK AUSUBEL; Fiscal Year: 1993
    ..cloned several Arabidopsis defense-related genes for use in monitoring the defense response including PAL1 (phenylalanine ammonia lyase), BGL1, BGL2, BGL3 (beta-1,3-glucanases), and GST1 (glutathione-S-transferase)...
  25. BETA2 INTEGRIN SIGNALING IN HUMAN NEUTROPHILS
    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...