Compounds > phenylalanine

phenylalanine and Tyrosinemias

phenylalanine has been researched along with Tyrosinemias in 30 studies

Research

Studies (30)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's3 (10.00)18.2507
2000's5 (16.67)29.6817
2010's15 (50.00)24.3611
2020's7 (23.33)2.80

Authors

AuthorsStudies
Arnoux, JB; Bou-Gharios, G; Davison, AS; Gallagher, JA; Hughes, AT; Imrich, R; M, K; Milan, AM; Norman, BP; Olsson, B; Ranganath, LR; Rudebeck, M1
Adam, S; Allen, H; Ash, J; Dale, C; Daly, A; Dixon, M; Dunlop, C; Ellerton, C; Evans, S; Firman, S; Ford, S; Freedman, F; Gribben, J; Howe, S; Khan, F; MacDonald, A; McDonald, J; McStravick, N; Nguyen, P; Oxley, N; Simpson, E; Skeath, R; Terry, A; White, L; Woodall, A1
Birkeland, E; Haavik, J; Kleppe, R; Patil, S; Szigetvari, PD1
Bou-Gharios, G; Gallagher, JA; Hughes, AT; Hughes, JH; Jarvis, JC; Judd, S; Milan, AM; Ranganath, LR; Sutherland, H; Wilson, PJM1
Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; Rodenburg, IL; Santra, S; van de Krogt, J; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ1
Bierau, J; Cassiman, D; Daly, A; De Laet, C; Eyskens, F; Gissen, P; Goyens, PJ; Huijbregts, SCJ; Jahja, R; Jameson, EA; MacDonald, A; McKiernan, PJ; Morris, AAM; Mumford, N; Rahman, Y; Timmer, C; van Ginkel, WG; van Hasselt, PM; van Spronsen, FJ; van Vliet, K; Vara, R; Wilcox, G1
Arias, C; Cabello, JF; Castro, G; Cornejo, V; De la Parra, A; Fuenzalida, K; Medina, MF; Valiente, A1
Bärhold, F; Beblo, S; Bogovic, N; Dahl, SV; Das, AM; Dokoupil, K; Fekete, A; Fischer, A; Freisinger, P; Heddrich-Ellerbrok, M; Hochuli, M; Jörg-Streller, M; Korpel, V; Lahl, J; Lier, D; Mayorandan, S; Meyer, U; Möslinger, D; Neugebauer, AK; Och, U; Rohde, C; Rosenbaum-Fabian, S; Thimm, EM; van Teeffelen-Heithoff, A1
Aldámiz-Echevarría, L; Andrade, F; Couce, ML; González-Lamuño, D; Sánchez-Pintos, P1
Bordugo, A; Camilot, M; Campostrini, N; Gugelmo, G; Ion-Popa, F; Maines, E; Pasini, A; Pietrobelli, A; Tadiotto, E; Teofoli, F; Vincenzi, M1
Huijbregts, SCJ; Jahja, R; van Ginkel, WG; van Spronsen, FJ1
Endo, F; Ito, M; Nakamura, K; Shigematsu, Y1
Das, AM; Meyer, U; van Rijn, M; van Spronsen, FJ1
Burgerhof, JGM; de Blaauw, P; Heiner-Fokkema, MR; Rubio Gozalbo, ME; van Ginkel, WG; van Spronsen, FJ; van Vliet, D1
Kassel, R; Rudnick, DA; Sprietsma, L1
Banerjee, P; Banik, D; Dutta, R; Kundu, S; Sarkar, N1
Carton, D; Dan, B; De Laet, C; François, B; Goyens, PJ; Jaeken, J; Munoz, VT; Sokal, EM1
Jitraruch, S; Leelaudomlipi, S; Sornmayura, P; Sriphojanart, S; Teeraratkul, S; Treepongkaruna, S; Viengteerawat, S; Wattanasirichaigoon, D1
Boenzi, S; Della Bona, ML; Dionisi-Vici, C; la Marca, G; Malvagia, S; Martinelli, D; Materazzi, S1
Chakrapani, A; Daly, A; Davies, P; Gokmen-Ozel, H; MacDonald, A; McKiernan, P; Preece, MA1
Biernacka, M; Biernacki, M; Jakubowska-Winecka, A; Kowalik, A; Kuśmierska, K; Pohorecka, M; Sykut-Cegielska, J1
Andersson, HC; Cunningham, A; Hooper, PF; Techakittiroj, C; Thoene, J1
Menkes, JH1
da Silva Ferreira, ME; Goldman, GH; Goldman, MH; Savoldi, M; Sueli Bonato, P1
Endo, F1
Clayton, PT; Leonard, JV; Van Wyk, KG; Wilson, CJ1
Anele, E; Becker, D; De Mari, J; Giugliani, R; Lewis, E; Neto, EC; Rubim, R; Schulte, J1
Al-Dhalimy, M; Finegold, M; Grompe, M; Overturf, K1
al-Essa, MA; Ozand, PT; Rashed, MS1

Reviews

3 review(s) available for phenylalanine and Tyrosinemias

ArticleYear
Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Humans; Nervous System Diseases; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Dietary Considerations in Tyrosinemia Type I.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Cyclohexanones; Diet; Humans; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
[Inborn errors of tyrosine metabolism: abnormalities in catabolic pathway of phenylalanine and tyrosine].
    Seikagaku. The Journal of Japanese Biochemical Society, 1999, Volume: 71, Issue:11

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acetoacetates; Amino Acid Metabolism, Inborn Errors; Animals; Humans; Mice; Mutation; Phenylalanine; Tyrosine; Tyrosinemias

1999

Trials

1 trial(s) available for phenylalanine and Tyrosinemias

ArticleYear
Neuropsychological outcome of NTBC-treated patients with tyrosinaemia type 1.
    Developmental medicine and child neurology, 2011, Volume: 53, Issue:10

    Topics: Adolescent; Belgium; Child; Child, Preschool; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Intelligence; Intelligence Tests; Male; Neuropsychological Tests; Nitrobenzoates; Phenylalanine; Treatment Outcome; Tyrosine; Tyrosinemias

2011

Other Studies

26 other study(ies) available for phenylalanine and Tyrosinemias

ArticleYear
Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.
    Scientific reports, 2022, 09-27, Volume: 12, Issue:1

    Topics: Alkaptonuria; Brain Diseases, Metabolic, Inborn; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Phenylalanine; Phenylpropionates; Tyrosine; Tyrosinemias

2022
UK Dietary Practices for Tyrosinaemias: Time for Change.
    Nutrients, 2022, Dec-07, Volume: 14, Issue:24

    Topics: Diet; Fruit; Phenylalanine; Tyrosinemias; United Kingdom; Vegetables

2022
The effects of phenylalanine and tyrosine levels on dopamine production in rat PC12 cells. Implications for treatment of phenylketonuria, tyrosinemia type 1 and comorbid neurodevelopmental disorders.
    Neurochemistry international, 2023, Volume: 171

    Topics: Animals; Dopamine; Neurodevelopmental Disorders; PC12 Cells; Phenylalanine; Phenylketonurias; Proteomics; Rats; Tyrosine; Tyrosine 3-Monooxygenase; Tyrosinemias

2023
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2

    Topics: Alkaptonuria; Animals; Cyclohexanones; Diet, Protein-Restricted; Female; Humans; Male; Mice; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2020
The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.
    Nutrients, 2019, Nov-18, Volume: 11, Issue:11

    Topics: Adolescent; Adult; Child; Cyclohexanones; Dietary Supplements; Enzyme Inhibitors; Female; Heptanoates; Humans; Linear Models; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias; Young Adult

2019
Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients.
    Orphanet journal of rare diseases, 2019, 12-04, Volume: 14, Issue:1

    Topics: Adolescent; Adult; Child; Cyclohexanones; Humans; Male; Nitrobenzoates; Phenylalanine; Quality of Life; Tyrosine; Tyrosinemias; Young Adult

2019
Case report: Maternal tyrosinemia type 1a under NTBC treatment with tyrosine- and phenylalanine restricted diet in Chile.
    American journal of medical genetics. Part C, Seminars in medical genetics, 2020, Volume: 184, Issue:4

    Topics: Adolescent; Chile; Diet; Female; Humans; Phenylalanine; Tyrosine; Tyrosinemias

2020
Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.
    Nutrients, 2020, Dec-31, Volume: 13, Issue:1

    Topics: Adolescent; Austria; Child; Child, Preschool; Combined Modality Therapy; Cyclohexanones; Diet, Protein-Restricted; Dietary Proteins; Enzyme Inhibitors; Female; Germany; Humans; Male; Nitrobenzoates; Patient Compliance; Phenylalanine; Practice Guidelines as Topic; Prospective Studies; Retrospective Studies; Surveys and Questionnaires; Switzerland; Treatment Outcome; Tyrosine; Tyrosinemias; Young Adult

2020
Treatment adherence in tyrosinemia type 1 patients.
    Orphanet journal of rare diseases, 2021, 06-03, Volume: 16, Issue:1

    Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Humans; Infant; Nitrobenzoates; Phenylalanine; Prognosis; Treatment Adherence and Compliance; Tyrosine; Tyrosinemias; Young Adult

2021
High-protein goat's milk diet identified through newborn screening: clinical warning of a potentially dangerous dietetic practice.
    Public health nutrition, 2017, Volume: 20, Issue:15

    Topics: Amino Acids; Animals; Diet; Dietary Proteins; Female; Goats; Humans; Infant Formula; Infant Nutritional Physiological Phenomena; Infant, Newborn; Methionine; Milk; Milk Proteins; Milk, Human; Neonatal Screening; Phenylalanine; Tyrosinemias

2017
Tyrosinemia Type I in Japan: A Report of Five Cases.
    Advances in experimental medicine and biology, 2017, Volume: 959

    Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Japan; Liver; Liver Transplantation; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2017
Presumptive brain influx of large neutral amino acids and the effect of phenylalanine supplementation in patients with Tyrosinemia type 1.
    PloS one, 2017, Volume: 12, Issue:9

    Topics: Adolescent; Amino Acids, Neutral; Brain; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Phenylalanine; Tyrosinemias

2017
Gene Editing Successfully Corrects 2 Amino Acid Disorders: In 2 preclinical studies using CRISPR-mediated gene editing, phenylketonuria and hereditary tyrosinemia type 1 were corrected.
    American journal of medical genetics. Part A, 2019, Volume: 179, Issue:1

    Topics: Amino Acids; Animals; CRISPR-Cas Systems; Disease Models, Animal; Gene Editing; Humans; Hydrolases; Mice; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Tyrosine; Tyrosinemias

2019
Pregnancy in an NTBC-treated patient with hereditary tyrosinemia type I.
    Journal of pediatric gastroenterology and nutrition, 2015, Volume: 60, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Combined Modality Therapy; Contraindications; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Phenylalanine; Pregnancy; Pregnancy Complications; Term Birth; Tyrosine; Tyrosinemias

2015
Investigation of Fibril Forming Mechanisms of l-Phenylalanine and l-Tyrosine: Microscopic Insight toward Phenylketonuria and Tyrosinemia Type II.
    The journal of physical chemistry. B, 2017, 02-23, Volume: 121, Issue:7

    Topics: Crown Ethers; Europium; Hydrogen Bonding; Kinetics; Macromolecular Substances; Phenylalanine; Phenylketonurias; Samarium; Terbium; Tyrosine; Tyrosinemias

2017
Long-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report.
    Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 2011, Volume: 94, Issue:10

    Topics: Asian People; Diet Therapy; Heptanoates; Humans; Hydrolases; Infant; Liver Failure; Liver Transplantation; Living Donors; Male; Mutation; Phenylalanine; Thailand; Treatment Outcome; Tyrosine; Tyrosinemias

2011
LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
    Analytical chemistry, 2012, Jan-17, Volume: 84, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Biomarkers; Child; Child, Preschool; Chromatography, Liquid; Cyclohexanones; Diet, Protein-Restricted; Drug Monitoring; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Kidney; Liver; Methionine; Nitrobenzoates; Phenylalanine; Tandem Mass Spectrometry; Tyrosine; Tyrosinemias

2012
Diurnal variation of phenylalanine concentrations in tyrosinaemia type 1: should we be concerned?
    Journal of human nutrition and dietetics : the official journal of the British Dietetic Association, 2012, Volume: 25, Issue:2

    Topics: Child; Child, Preschool; Circadian Rhythm; Dietary Proteins; Female; Humans; Male; Phenylalanine; Practice Guidelines as Topic; Retrospective Studies; Tyrosine; Tyrosinemias

2012
Behavioral and intellectual functioning in patients with tyrosinemia type I.
    Pediatric endocrinology, diabetes, and metabolism, 2012, Volume: 18, Issue:3

    Topics: Adolescent; Attention Deficit Disorder with Hyperactivity; Child; Cognition Disorders; Female; Humans; Intelligence Tests; Male; Mental Disorders; Phenylalanine; Problem Solving; Tyrosine; Tyrosinemias; Wechsler Scales

2012
High protein diet mimics hypertyrosinemia in newborn infants.
    The Journal of pediatrics, 2005, Volume: 146, Issue:2

    Topics: Diagnosis, Differential; Diet Therapy; Dietary Proteins; Female; Humans; Infant Food; Infant, Newborn; Male; Phenylalanine; Phenylketonurias; Treatment Outcome; Tyrosine; Tyrosinemias

2005
The history of hypertyrosinemia caused by high protein diets.
    The Journal of pediatrics, 2006, Volume: 148, Issue:2

    Topics: Dietary Proteins; Humans; Infant Food; Infant, Newborn; Intellectual Disability; Phenylalanine; Tyrosine; Tyrosinemias

2006
Fungal metabolic model for tyrosinemia type 3: molecular characterization of a gene encoding a 4-hydroxy-phenyl pyruvate dioxygenase from Aspergillus nidulans.
    Eukaryotic cell, 2006, Volume: 5, Issue:8

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Aspergillus nidulans; Gene Deletion; Humans; Models, Biological; Phenylalanine; Phenylpyruvic Acids; Tyrosine; Tyrosinemias

2006
Phenylalanine supplementation improves the phenylalanine profile in tyrosinaemia.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:7

    Topics: Child; Child, Preschool; Dietary Supplements; Humans; Infant; Phenylalanine; Treatment Outcome; Tyrosine; Tyrosinemias

2000
Persistent tyrosinemia detected by thin-layer chromatography.
    The Southeast Asian journal of tropical medicine and public health, 1999, Volume: 30 Suppl 2

    Topics: Chromatography, Thin Layer; Humans; Infant, Newborn; Neonatal Screening; Phenylalanine; Tyrosine; Tyrosinemias

1999
Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I.
    Molecular genetics and metabolism, 2002, Volume: 75, Issue:1

    Topics: Animals; Cyclohexanones; Diet; Disease Models, Animal; Enzyme Inhibitors; Hydrolases; Mice; Mice, Knockout; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias

2002
Tyrosinaemia type II: an easily diagnosed metabolic disorder with a rewarding therapeutic response.
    Eastern Mediterranean health journal = La revue de sante de la Mediterranee orientale = al-Majallah al-sihhiyah li-sharq al-mutawassit, 1999, Volume: 5, Issue:6

    Topics: Adult; Child; Chromatography, High Pressure Liquid; Female; Humans; Intellectual Disability; Male; Mass Spectrometry; Phenylalanine; Retrospective Studies; Tyrosine; Tyrosinemias

1999