glutamic acid and BH4 Deficiency

glutamic acid has been researched along with BH4 Deficiency in 13 studies

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19904 (30.77)18.7374
1990's3 (23.08)18.2507
2000's5 (38.46)29.6817
2010's1 (7.69)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Binz, PA; Fabritz, S; Griss, R; Haas, D; Hiblot, J; Johnsson, K; Okun, JG; Roux, C; Xue, L; Yu, Q1
BESSMAN, SP; MEISTER, A; UDENFRIEND, S1
VULLIAMY, DG; WOOLF, LI1
Matalon, R; Michals-Matalon, K; Rady, PL; Surendran, S; Szucs, S; Tyring, SK1
Gu, XF; Zhang, H1
Dennis, DM; Glushakov, AV; Laipis, PJ; Martynyuk, AE; Seubert, CN; Sumners, C1
Levy, HL1
Abadie, V; Berthelon, M; Jaruzelska, J; Lyonnet, S; Millasseau, P; Munnich, A; Rey, F; Rey, J1
Debreceni, B; Farkas, V; Fischer, GM; Laszlo, A; Nemeti, B; Sandor, A; Schaffer, Z; Somogyi, C1
Konecki, DS; Lichter-Konecki, U; Schlotter, M; Trefz, FK1
Eisensmith, RC; Güttler, F; Okano, Y; Wang, T; Woo, SL1
Bell, EF; Brummel, MC; Filer, LJ; Krause, WL; Stegink, LD; Wolf-Novak, LC; Ziegler, EE1

Reviews

1 review(s) available for glutamic acid and BH4 Deficiency

ArticleYear
Impaired glutamatergic synaptic transmission in the PKU brain.
    Molecular genetics and metabolism, 2005, Volume: 86 Suppl 1

    Topics: Animals; Brain; Cells, Cultured; gamma-Aminobutyric Acid; Glutamic Acid; Mice; Neurons; Phenylalanine; Phenylketonurias; Rats; Receptors, AMPA; Receptors, Glutamate; Receptors, N-Methyl-D-Aspartate; Synaptic Transmission

2005

Trials

1 trial(s) available for glutamic acid and BH4 Deficiency

ArticleYear
Aspartame-sweetened beverage: effect on plasma amino acid concentrations in normal adults and adults heterozygous for phenylketonuria.
    The Journal of nutrition, 1987, Volume: 117, Issue:11

    Topics: Adult; Amino Acids; Asparagine; Aspartame; Aspartic Acid; Beverages; Biological Transport; Dipeptides; Female; Glutamates; Glutamic Acid; Glutamine; Heterozygote; Humans; Kinetics; Male; Phenylalanine; Phenylketonurias; Tyrosine

1987

Other Studies

11 other study(ies) available for glutamic acid and BH4 Deficiency

ArticleYear
Semisynthetic sensor proteins enable metabolic assays at the point of care.
    Science (New York, N.Y.), 2018, 09-14, Volume: 361, Issue:6407

    Topics: Bioluminescence Resonance Energy Transfer Techniques; Biosensing Techniques; Blood Glucose; Escherichia coli Proteins; Glutamic Acid; Humans; Monitoring, Physiologic; NADP; Oxidation-Reduction; Phenylalanine; Phenylketonurias; Point-of-Care Testing; Tetrahydrofolate Dehydrogenase

2018
Diminished phenylketonuria in phenylpyruvic oligophrenia after administration of L-glutamine, L-glutamate or L-asparagine.
    The Journal of clinical investigation, 1956, Volume: 35, Issue:6

    Topics: Asparagine; Body Fluids; Glutamic Acid; Glutamine; Humans; Intellectual Disability; Ketones; Phenylketonurias; Urine

1956
Phenylketonuria with a study of the effect upon it of glutamic acid.
    Archives of disease in childhood, 1951, Volume: 26, Issue:130

    Topics: Glutamates; Glutamic Acid; Humans; Intellectual Disability; Phenylketonurias

1951
High level of orexin A observed in the phenylketonuria mouse brain is due to the abnormal expression of prepro-orexin.
    Biochemical and biophysical research communications, 2004, Apr-30, Volume: 317, Issue:2

    Topics: Animals; Brain; Carrier Proteins; gamma-Aminobutyric Acid; Gene Expression Profiling; Gene Expression Regulation; Glutamic Acid; Intracellular Signaling Peptides and Proteins; Mice; Neuropeptides; Oligonucleotide Array Sequence Analysis; Orexins; Phenylalanine; Phenylketonurias; Signal Transduction

2004
A study of gene expression profiles of cultured embryonic rat neurons induced by phenylalanine.
    Metabolic brain disease, 2005, Volume: 20, Issue:1

    Topics: Animals; Cells, Cultured; Cerebral Cortex; Gene Expression Profiling; Gene Expression Regulation; Glutamic Acid; Neurons; Neurotoxins; Oligonucleotide Array Sequence Analysis; Phenylalanine; Phenylketonurias; Rats; RNA, Messenger; Signal Transduction; Up-Regulation

2005
Metabolic disorders in the center of genetic medicine.
    The New England journal of medicine, 2005, Nov-03, Volume: 353, Issue:18

    Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases, Metabolic, Inborn; Glutamate-Ammonia Ligase; Glutamic Acid; Glutamine; Homocystinuria; Humans; Hyperhomocysteinemia; Infant, Newborn; Mutation; Phenylketonurias

2005
Illegitimate transcription of the phenylalanine hydroxylase gene in lymphocytes for identification of mutations in phenylketonuria.
    Human molecular genetics, 1993, Volume: 2, Issue:1

    Topics: Amino Acid Sequence; Base Sequence; Codon; DNA; Exons; Glutamates; Glutamic Acid; Glycine; Humans; Infant; Leucine; Liver; Lymphocytes; Male; Molecular Sequence Data; Oligodeoxyribonucleotides; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Point Mutation; Polymorphism, Restriction Fragment Length; Transcription, Genetic

1993
Metabolism of carnitine in phenylacetic acid-treated rats and in patients with phenylketonuria.
    Biochimica et biophysica acta, 2000, Jun-15, Volume: 1501, Issue:2-3

    Topics: Adult; Animals; Betaine; Carnitine; Female; Glutamic Acid; Homogentisic Acid; Humans; Ketoglutaric Acids; Liver; Male; Mass Spectrometry; Phenylacetates; Phenylketonurias; Phenylpyruvic Acids; Rats; Rats, Wistar

2000
The identification of two mis-sense mutations at the PAH gene locus in a Turkish patient with phenylketonuria.
    Human genetics, 1991, Volume: 87, Issue:4

    Topics: Alleles; Base Sequence; Chromosome Mapping; DNA; DNA Mutational Analysis; Glutamates; Glutamic Acid; Glycine; Homozygote; Humans; Molecular Sequence Data; Mutation; Nucleic Acid Hybridization; Phenylalanine Hydroxylase; Phenylketonurias; Polymerase Chain Reaction; Polymorphism, Restriction Fragment Length; Turkey

1991
Recurrent mutation in the human phenylalanine hydroxylase gene.
    American journal of human genetics, 1990, Volume: 46, Issue:5

    Topics: Alleles; Base Sequence; DNA; Female; Genes; Genetic Carrier Screening; Glutamates; Glutamic Acid; Haplotypes; Humans; Leukocytes; Lysine; Male; Molecular Sequence Data; Mutation; Oligonucleotide Probes; Pedigree; Phenylalanine Hydroxylase; Phenylketonurias; Polymerase Chain Reaction; Polymorphism, Restriction Fragment Length; Restriction Mapping

1990
Aspartame. Review of safety issues. Council on Scientific Affairs.
    JAMA, 1985, Jul-19, Volume: 254, Issue:3

    Topics: Adolescent; Adult; Animals; Aspartame; Aspartic Acid; Brain Chemistry; Brain Diseases; Brain Neoplasms; Carbonated Beverages; Carcinogens; Child; Dietary Carbohydrates; Diketopiperazines; Dipeptides; Drug Stability; Endocrine System Diseases; Female; Glutamates; Glutamic Acid; Humans; Infant; Intellectual Disability; Methanol; Mice; Neurotransmitter Agents; Phenylalanine; Phenylketonurias; Piperazines; Pregnancy; Rats

1985