homogentisic acid and nitisinone

homogentisic acid has been researched along with nitisinone in 24 studies

Research

Studies (24)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (4.17)18.2507
2000's5 (20.83)29.6817
2010's12 (50.00)24.3611
2020's6 (25.00)2.80

Authors

AuthorsStudies
Maeda, Y; Oda, K; Suzuki, T; Suzuki, Y; Yoshikawa, Y1
Lorenzini, S; Mannoni, A; Selvi, E1
Al-Dhalimy, M; Finegold, M; Grompe, M; Groopman, J; Iordanov, MS; Ou, CN; Ryabinina, O; van Den Berg, IE; Vogel, A1
Berger, R; Grompe, M; Jacobs, SM; Klomp, LW; Luijerink, MC; Malingré, HE; van Beurden, EA; van den Berg, IE1
Bernardini, I; Gahl, WA; Gerber, LH; Kaiser-Kupfer, MI; O'Brien, K; Perry, MB; Rubin, BI; Sebring, N; Suwannarat, P; Tsilou, E1
Berger, R; Jacobs, SM; Klomp, LW; van Beurden, DH; van den Berg, IE1
Anolik, R; Pomeranz, MK; Yancovitz, M1
Bernardini, I; Bryant, J; Gahl, WA; Introne, WJ; Kayser, MA; Moylan, E; O'Brien, KE; Perry, MB; Reynolds, JC; Sachdev, V; Suwannarat, P; Troendle, J; Tsilou, E1
Cox, TF; Ranganath, L1
Cox, TF; Ranganath, LR1
Ayoob, H; Braconi, D; Briggs, MC; Bygott, H; Christensen, P; Cox, TF; Cronlund, A; Dutton, JJ; Fitzgerald, R; Gallagher, JA; Genovese, F; Hall, AK; Hughes, AT; Imrich, R; Jarvis, JC; Junestrand, C; Kullenberg, T; Laan, D; Le Quan Sang, KH; McCaffrey, J; Milan, AM; Nemethova, M; Olsson, B; Psarelli, EE; Ranganath, LR; Ross, G; Rovensky, J; Rudebeck, M; Santucci, A; Sireau, N; Svensson, L; Szamosi, J; Timmis, OG; van Kan, C; Zatkova, A1
Christensen, P; Davison, AS; Dutton, JJ; Gallagher, JA; Hughes, AT; Milan, AM; Ranganath, LR; Ross, G1
Davison, AS; Gallagher, JA; Milan, AM; Ranganath, LR1
Bernardini, G; Botta, M; Braconi, D; Dreassi, E; Geminiani, M; Laschi, M; Manetti, F; Marzocchi, B; Millucci, L; Santucci, A1
Curtis, S; Davison, AS; Devine, J; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Ranganath, LR; Usher, J1
Barton, GJ; Briggs, M; Cox, TF; Daroszewska, A; Davison, AS; Devine, JM; Dillon, JP; Fisher, M; Gallagher, JA; Griffin, R; Harrold, J; Hughes, AT; Hughes, G; Jarvis, JC; Jones, A; Judd, S; Khedr, M; Loftus, N; McCormick, M; Milan, AM; Psarelli, EE; Ranganath, LR; Sireau, N; Taylor, S; Usher, JL; Vinjamuri, S; West, E1
Adam, D1
Cox, TF; Davison, AS; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovensky, J1
Davison, AS; Gallagher, JA; Hughes, AT; Milan, AM; Ranganath, LR; Sireau, N1
Arnoux, JB; Braconi, D; Briggs, M; Bröijersén, A; Bygott, H; Cox, TF; Davison, AS; Dillon, JP; Fisher, M; FitzGerald, R; Gallagher, JA; Genovese, F; Glasova, H; Hall, AK; Hughes, AT; Hughes, JH; Imrich, R; Jarvis, JC; Khedr, M; Laan, D; Le Quan Sang, KH; Loftus, N; Luangrath, E; Lukáčová, O; Milan, AM; Mistry, A; Mlynáriková, V; Norman, BP; Olsson, B; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovenský, J; Rudebeck, M; Santucci, A; Scott, C; Sedláková, J; Shweihdi, E; Sireau, N; Stančík, R; Szamosi, J; Taylor, S; van Kan, C; Vinjamuri, S; Vrtíková, E; Webb, C; West, E; Záňová, E; Zatkova, A1
Gallagher, JA; Khedr, M; Ranganath, LR; Vinjamuri, S1
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
Havranova, A; Imrich, R; Lukacova, O; Penesova, A; Radikova, Z; Ranganath, L; Sedlakova, J; Vlcek, M; Zanova, E; Zatkova, A1
Bou-Gharios, G; Cooper, IR; Gallagher, JA; Norman, B; Ooi, N; Ranganath, LR; Savage, VJ; Sireau, N1

Reviews

2 review(s) available for homogentisic acid and nitisinone

ArticleYear
Acute fatal metabolic complications in alkaptonuria.
    Journal of inherited metabolic disease, 2016, Volume: 39, Issue:2

    Topics: Acute Disease; Alkaptonuria; Cyclohexanones; Erythrocytes; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Metabolic Diseases; Nitrobenzoates; Oxidation-Reduction

2016
Alkaptonuria - Many questions answered, further challenges beckon.
    Annals of clinical biochemistry, 2020, Volume: 57, Issue:2

    Topics: Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Homogentisic Acid; Humans; Mice; Nitrobenzoates; Tyrosine

2020

Trials

5 trial(s) available for homogentisic acid and nitisinone

ArticleYear
A 3-year randomized therapeutic trial of nitisinone in alkaptonuria.
    Molecular genetics and metabolism, 2011, Volume: 103, Issue:4

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adult; Alkaptonuria; Cyclohexanones; Homogentisic Acid; Humans; Middle Aged; Nitrobenzoates; Prospective Studies; Tyrosine

2011
Suitability Of Nitisinone In Alkaptonuria 1 (SONIA 1): an international, multicentre, randomised, open-label, no-treatment controlled, parallel-group, dose-response study to investigate the effect of once daily nitisinone on 24-h urinary homogentisic acid
    Annals of the rheumatic diseases, 2016, Volume: 75, Issue:2

    Topics: Adult; Alkaptonuria; Cyclohexanones; Dose-Response Relationship, Drug; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Research Design; Tyrosine

2016
Quantification of the flux of tyrosine pathway metabolites during nitisinone treatment of Alkaptonuria.
    Scientific reports, 2019, 07-11, Volume: 9, Issue:1

    Topics: Adult; Alkaptonuria; Cyclohexanones; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Pigments, Biological; Tandem Mass Spectrometry; Tyrosine

2019
Efficacy and safety of once-daily nitisinone for patients with alkaptonuria (SONIA 2): an international, multicentre, open-label, randomised controlled trial.
    The lancet. Diabetes & endocrinology, 2020, Volume: 8, Issue:9

    Topics: Adult; Aged; Alkaptonuria; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Internationality; Longitudinal Studies; Male; Middle Aged; Nitrobenzoates; Single-Blind Method; Treatment Outcome

2020
Evaluation of Homogentisic Acid, a Prospective Antibacterial Agent Highlighted by the Suitability of Nitisinone in Alkaptonuria 2 (SONIA 2) Clinical Trial.
    Cells, 2023, Jun-21, Volume: 12, Issue:13

    Topics: Alkaptonuria; Anti-Bacterial Agents; Gram-Negative Bacteria; Gram-Positive Bacteria; Homogentisic Acid; Humans; Prospective Studies

2023

Other Studies

17 other study(ies) available for homogentisic acid and nitisinone

ArticleYear
A novel therapeutic trial of homogentisic aciduria in a murine model of alkaptonuria.
    Journal of human genetics, 1999, Volume: 44, Issue:2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Homogentisic Acid; Mice; Mice, Mutant Strains; Nitrobenzoates

1999
Alkaptonuria.
    The New England journal of medicine, 2003, 04-03, Volume: 348, Issue:14

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Antioxidants; Ascorbic Acid; Benzoquinones; Cyclohexanones; Enzyme Inhibitors; Homogentisic Acid; Humans; Nitrobenzoates; Oxidation-Reduction

2003
Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death.
    Hepatology (Baltimore, Md.), 2004, Volume: 39, Issue:2

    Topics: Acetaminophen; Analgesics, Non-Narcotic; Animals; Apoptosis; Cell Survival; Central Nervous System Depressants; Chronic Disease; Cyclohexanones; Enzyme Inhibitors; Ethanol; Fas Ligand Protein; fas Receptor; Glutathione; Homogentisic Acid; HSP70 Heat-Shock Proteins; Hydrolases; Liver Failure, Acute; Membrane Glycoproteins; Mice; Mice, Mutant Strains; Necrosis; NF-kappa B; Nitrobenzoates; Oxidative Stress; Proto-Oncogene Proteins c-jun; Tyrosinemias; Up-Regulation

2004
Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1.
    Kidney international, 2004, Volume: 66, Issue:3

    Topics: Animals; Caspase 3; Caspase 9; Caspases; Cyclohexanones; Disease Models, Animal; DNA Fragmentation; Enzyme Inhibitors; Hepatocytes; Homogentisic Acid; Hydrolases; In Situ Nick-End Labeling; Kidney Tubules, Proximal; Mice; Mice, Knockout; Nitrobenzoates; Survival Rate; Tyrosine; Tyrosinemias

2004
Use of nitisinone in patients with alkaptonuria.
    Metabolism: clinical and experimental, 2005, Volume: 54, Issue:6

    Topics: Adult; Aged; Alkaptonuria; Cyclohexanones; Dietary Proteins; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Tyrosine

2005
Kidneys of mice with hereditary tyrosinemia type I are extremely sensitive to cytotoxicity.
    Pediatric research, 2006, Volume: 59, Issue:3

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cyclohexanones; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Hydrolases; In Situ Nick-End Labeling; Kidney; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Nitrobenzoates; Tyrosinemias

2006
Alkaptonuria.
    Dermatology online journal, 2010, Nov-15, Volume: 16, Issue:11

    Topics: Aged; Alkaptonuria; Arthritis; Cyclohexanones; Female; Homogentisic Acid; Humans; Joint Diseases; Nitrobenzoates; Ochronosis; Pigmentation Disorders

2010
A quantitative assessment of alkaptonuria: testing the reliability of two disease severity scoring systems.
    Journal of inherited metabolic disease, 2011, Volume: 34, Issue:6

    Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Alkaptonuria; Causality; Cohort Studies; Comorbidity; Cyclohexanones; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Hypertension; Male; Middle Aged; Nitrobenzoates; Ochronosis; Pain; Regression Analysis; Reproducibility of Results; Severity of Illness Index; Sex Distribution; Smoking; Surveys and Questionnaires

2011
Natural history of alkaptonuria revisited: analyses based on scoring systems.
    Journal of inherited metabolic disease, 2011, Volume: 34, Issue:6

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Adult; Aged; Aged, 80 and over; Alkaptonuria; Arthralgia; Arthritis; Cartilage, Articular; Causality; Cohort Studies; Comorbidity; Cyclohexanones; Disease Progression; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Ochronosis; Pain; Surveys and Questionnaires

2011
Serum markers in alkaptonuria: simultaneous analysis of homogentisic acid, tyrosine and nitisinone by liquid chromatography tandem mass spectrometry.
    Annals of clinical biochemistry, 2015, Volume: 52, Issue:Pt 5

    Topics: Alkaptonuria; Biomarkers; Chromatography, Liquid; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine

2015
Inhibition of para-Hydroxyphenylpyruvate Dioxygenase by Analogues of the Herbicide Nitisinone As a Strategy to Decrease Homogentisic Acid Levels, the Causative Agent of Alkaptonuria.
    ChemMedChem, 2016, Apr-05, Volume: 11, Issue:7

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Cell Survival; Cyclohexanones; Dose-Response Relationship, Drug; Herbicides; Homogentisic Acid; Humans; Male; Molecular Structure; Nitrobenzoates; Rats; Rats, Wistar; Structure-Activity Relationship

2016
The effect of nitisinone on homogentisic acid and tyrosine: a two-year survey of patients attending the National Alkaptonuria Centre, Liverpool.
    Annals of clinical biochemistry, 2017, Volume: 54, Issue:3

    Topics: Adult; Aged; Alkaptonuria; Chromatography, Liquid; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Longitudinal Studies; Male; Middle Aged; Monitoring, Physiologic; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine; United Kingdom

2017
Nitisinone arrests ochronosis and decreases rate of progression of Alkaptonuria: Evaluation of the effect of nitisinone in the United Kingdom National Alkaptonuria Centre.
    Molecular genetics and metabolism, 2018, Volume: 125, Issue:1-2

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Cyclohexanones; Disease Progression; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Ochronosis; United Kingdom

2018
A father's fight to help his sons - and fix clinical trials.
    Nature, 2019, Volume: 565, Issue:7738

    Topics: Adolescent; Alkaptonuria; Animals; Autopsy; Child; Clinical Trials as Topic; Compassionate Use Trials; Cyclohexanones; Disease Models, Animal; Disease Progression; Drug Approval; Endpoint Determination; Female; Homogentisic Acid; Humans; Infant; Male; Mice; National Health Programs; National Institutes of Health (U.S.); Nitrobenzoates; Observational Studies as Topic; Off-Label Use; Orphan Drug Production; Rare Diseases; Rats; Sample Size; Tyrosine; Tyrosinemias; United States; United States Food and Drug Administration

2019
Characterizing the alkaptonuria joint and spine phenotype and assessing the effect of homogentisic acid lowering therapy in a large cohort of 87 patients.
    Journal of inherited metabolic disease, 2021, Volume: 44, Issue:3

    Topics: Aged; Alkaptonuria; Cohort Studies; Cyclohexanones; Female; Homogentisic Acid; Humans; Joints; Linear Models; Male; Middle Aged; Nitrobenzoates; Ochronosis; Phenotype; Positron Emission Tomography Computed Tomography; Severity of Illness Index; Spine; United Kingdom

2021
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
Nutritional interventions for patients with alkaptonuria: A minireview.
    Endocrine regulations, 2023, Jan-01, Volume: 57, Issue:1

    Topics: Alkaptonuria; Homogentisic Acid; Humans; Ochronosis; Tyrosine; Tyrosinemias

2023