Page last updated: 2024-09-03

nitisinone and Disease Models, Animal

nitisinone has been researched along with Disease Models, Animal in 25 studies

Research

Studies (25)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's5 (20.00)18.2507
2000's5 (20.00)29.6817
2010's12 (48.00)24.3611
2020's3 (12.00)2.80

Authors

AuthorsStudies
Abrams, RPM; Bachani, M; Balasubramanian, A; Brimacombe, K; Dorjsuren, D; Eastman, RT; Hall, MD; Jadhav, A; Lee, MH; Li, W; Malik, N; Nath, A; Padmanabhan, R; Simeonov, A; Steiner, JP; Teramoto, T; Yasgar, A; Zakharov, AV1
Davison, AS; Gallagher, JA; Hughes, AT; Milan, AM; Ranganath, LR; Sireau, N1
Branson, S; Casimir, G; Claes, P; Colemonts-Vroninks, H; De Kock, J; Goyens, P; Marcelis, L; Martens, GA; Neuckermans, J; Vanhaecke, T1
Abu-Asab, MS; Adams, DR; Aderemi, JO; Albert, JS; Alur, RP; Bernardini, IM; Brooks, BP; Chan, CC; Cogliati, T; Megan, LH; Melch, MG; Onojafe, IF1
Adam, D1
Gallagher, JA; Jarvis, JC; Keenan, CM; Preston, AJ; Ranganath, LR; Sutherland, H; Taylor, AM; Williams, DP; Wilson, PJ; Wlodarski, B1
Qi, Z; Sun, R; Tian, Z; Wang, X; Wei, H1
Gallagher, JA; Ranganath, LR; Timmis, OG1
Gao, X; Li, L; Qi, Z; Sun, R; Tian, Z; Wang, X; Wei, H; Zeng, Z1
Barnby, E; Coker, SB; Hillgartner, MA; Koenig, AE; MacGregor, GG; Moore, ME1
Amiot, B; Chen, H; DeGrado, TR; Elgilani, F; Freeman, BL; Glorioso, J; Grompe, M; Harding, CO; Hickey, RD; Ho, CS; Ibrahim, SH; Ikeda, Y; Jiang, H; Lillegard, JB; Mao, SA; Marler, R; Nyberg, SL; O'Connor, MK; Peng, KW; Rinaldo, P; Russell, SJ; Suksanpaisan, L1
Barzi, M; Bissig, KD; Bissig-Choisat, B; Borowiak, M; Elsea, SH; Hubert, L; Legras, X; Mi, T; Pankowicz, FP; Ravishankar, M; Sumazin, P; Sun, Q; Tomolonis, JA; Yang, D1
Finegold, M; Grompe, M; Lee, AY; Rothfuss, A; Sharma, AD; Vogel, A; Wang, Z; Willenbring, H1
Dahlke, MH; Doenecke, A; Eggenhofer, E; Geissler, EK; Piso, P; Popp, FC; Renner, P; Schlitt, HJ; Slowik, P1
Bleidissel, M; Cantz, T; Gentile, L; Greber, B; Han, DW; Liu, N; Ott, M; Rittelmeyer, I; Rudolph, C; Schambach, A; Schöler, HR; Sgodda, M; Sharma, AD; Steinemann, D; Wu, G; Zaehres, H1
Adams, DR; Alur, RP; Bernardini, IM; Brilliant, MH; Brooks, BP; Chan, CC; Dolinska, MB; Gahl, WA; Onojafe, IF; Sergeev, YV; Simeonov, DR; Zhang, J1
Berger, R; Grompe, M; Jacobs, SM; Klomp, LW; Luijerink, MC; Malingré, HE; van Beurden, EA; van den Berg, IE1
Bergeron, A; Finegold, MJ; Jorquera, R; Langlois, C; Orejuela, D; Rhead, WJ; Tanguay, RM1
Bergeron, A; Finegold, MJ; Jorquera, R; Orejuela, D; Tanguay, RM1
al-Dhalimy, M; Finegold, M; Grompe, M; Kennaway, NG; Lindstedt, S; Ou, CN; Papaconstantinou, J; Torres-Ramos, CA1
Al-Dhalimy, M; Brantly, M; Finegold, M; Grompe, M; Ou, CN; Overturf, K; Tanguay, R1
Holme, E; Lindstedt, S1
Al-Dhalimy, M; Finegold, M; Grompe, M; Manning, K; Ou, CN; Overturf, K1
Maeda, Y; Oda, K; Suzuki, T; Suzuki, Y; Yoshikawa, Y1
Al-Dhalimy, M; Finegold, M; Grompe, M; Overturf, K1

Reviews

2 review(s) available for nitisinone and Disease Models, Animal

ArticleYear
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
Diagnosis and management of tyrosinemia type I.
    Current opinion in pediatrics, 1995, Volume: 7, Issue:6

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genetic Therapy; Humans; Liver Transplantation; Nitrobenzoates; Phenotype; Tyrosine

1995

Other Studies

23 other study(ies) available for nitisinone and Disease Models, Animal

ArticleYear
Therapeutic candidates for the Zika virus identified by a high-throughput screen for Zika protease inhibitors.
    Proceedings of the National Academy of Sciences of the United States of America, 2020, 12-08, Volume: 117, Issue:49

    Topics: Animals; Antiviral Agents; Artificial Intelligence; Chlorocebus aethiops; Disease Models, Animal; Drug Evaluation, Preclinical; High-Throughput Screening Assays; Immunocompetence; Inhibitory Concentration 50; Methacycline; Mice, Inbred C57BL; Protease Inhibitors; Quantitative Structure-Activity Relationship; Small Molecule Libraries; Vero Cells; Zika Virus; Zika Virus Infection

2020
Oxidative Stress, Glutathione Metabolism, and Liver Regeneration Pathways Are Activated in Hereditary Tyrosinemia Type 1 Mice upon Short-Term Nitisinone Discontinuation.
    Genes, 2020, 12-22, Volume: 12, Issue:1

    Topics: Animals; Cyclohexanones; Disease Models, Animal; Glutathione; Humans; Hydrolases; Liver; Liver Regeneration; Medication Adherence; Mice; Mice, Knockout; NF-E2-Related Factor 2; Nitrobenzoates; Oxidative Stress; Oxidoreductases; Signal Transduction; Tyrosine; Tyrosinemias; Withholding Treatment

2020
Minimal Efficacy of Nitisinone Treatment in a Novel Mouse Model of Oculocutaneous Albinism, Type 3.
    Investigative ophthalmology & visual science, 2018, 10-01, Volume: 59, Issue:12

    Topics: Administration, Oral; Albinism, Oculocutaneous; Animals; Blotting, Western; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genotyping Techniques; Melanins; Melanosomes; Membrane Glycoproteins; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Mice, Inbred DBA; Microscopy, Electron, Transmission; Nitrobenzoates; Oxidoreductases; Real-Time Polymerase Chain Reaction; Treatment Outcome; Tyrosine

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
Ochronotic osteoarthropathy in a mouse model of alkaptonuria, and its inhibition by nitisinone.
    Annals of the rheumatic diseases, 2014, Volume: 73, Issue:1

    Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Chondrocytes; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Female; Humans; Joint Diseases; Knee Joint; Male; Mice; Mice, Inbred BALB C; Mice, Knockout; Nitrobenzoates; Ochronosis

2014
Infiltrating neutrophils aggravate metabolic liver failure in fah-deficient mice.
    Liver international : official journal of the International Association for the Study of the Liver, 2015, Volume: 35, Issue:3

    Topics: Animals; Bilirubin; Body Weight; Cyclohexanones; Disease Models, Animal; Hepatocytes; Hydrolases; Inflammation; Interleukin-22; Interleukins; Liver; Liver Failure; Male; Mice; Mice, Knockout; Neutrophils; Nitrobenzoates; Receptors, CCR2; Tyrosine

2015
Progress in Alkaptonuria--are we near to an effective therapy?
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:5

    Topics: Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Genetic Therapy; Humans; Mice; Nitrobenzoates; Ochronosis; Treatment Outcome

2015
Natural Killer Cells-Produced IFN-γ Improves Bone Marrow-Derived Hepatocytes Regeneration in Murine Liver Failure Model.
    Scientific reports, 2015, Sep-08, Volume: 5

    Topics: Animals; Bone Marrow Cells; Bone Marrow Transplantation; Cell Fusion; Cyclohexanones; Disease Models, Animal; Female; Hepatocytes; Hydrolases; Interferon gamma Receptor; Interferon-gamma; Killer Cells, Natural; Liver Failure; Liver Regeneration; Male; Mice; Mice, Knockout; Monocytes; Nitrobenzoates; Protein Binding; Receptors, Interferon

2015
Tyrosinemia type I and not treatment with NTBC causes slower learning and altered behavior in mice.
    Journal of inherited metabolic disease, 2016, Volume: 39, Issue:5

    Topics: Animals; Behavior, Animal; Cyclohexanones; Disease Models, Animal; Hydrolases; Learning; Memory, Long-Term; Mice; Nitrobenzoates; Tyrosine; Tyrosinemias

2016
Curative ex vivo liver-directed gene therapy in a pig model of hereditary tyrosinemia type 1.
    Science translational medicine, 2016, 07-27, Volume: 8, Issue:349

    Topics: Animals; Cyclohexanones; Disease Models, Animal; Genetic Therapy; Hepatocytes; Hydrolases; Immunohistochemistry; Liver; Nitrobenzoates; Swine; Transplantation, Homologous; Tyrosinemias

2016
Reprogramming metabolic pathways in vivo with CRISPR/Cas9 genome editing to treat hereditary tyrosinaemia.
    Nature communications, 2016, 08-30, Volume: 7

    Topics: Animals; Cell Line; Cell Proliferation; CRISPR-Cas Systems; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Exons; Gene Editing; Genetic Therapy; Hepatocytes; Humans; Hydrolases; Liver; Metabolic Networks and Pathways; Mice; Mice, Inbred Strains; Mice, Knockout; Nitrobenzoates; Oxidoreductases; Phenotype; Tyrosinemias

2016
Loss of p21 permits carcinogenesis from chronically damaged liver and kidney epithelial cells despite unchecked apoptosis.
    Cancer cell, 2008, Jul-08, Volume: 14, Issue:1

    Topics: Animals; Apoptosis; Carcinoma, Hepatocellular; Cell Cycle; Cell Proliferation; Cyclin-Dependent Kinase Inhibitor p21; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Hepatectomy; Hepatocytes; Hydrolases; Kidney Diseases, Cystic; Kidney Neoplasms; Kidney Tubules, Proximal; Liver Neoplasms; Liver Regeneration; Mice; Mice, Knockout; Neoplasms; Nitrobenzoates; Tyrosinemias

2008
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice.
    Journal of gastroenterology and hepatology, 2010, Volume: 25, Issue:5

    Topics: Animals; Bilirubin; Biomarkers; Cell Proliferation; Cyclohexanones; Dependovirus; Disease Models, Animal; Genetic Therapy; Genetic Vectors; Hepatocytes; Hydrolases; Injections, Intravenous; L-Lactate Dehydrogenase; Liver; Liver Function Tests; Mice; Mice, Inbred BALB C; Mice, Knockout; Nitrobenzoates; Plasmids; Serum Albumin; Tail; Time Factors; Transfection; Tyrosinemias; Urea

2010
Generation of healthy mice from gene-corrected disease-specific induced pluripotent stem cells.
    PLoS biology, 2011, Volume: 9, Issue:7

    Topics: Animals; Cell Survival; Cells, Cultured; Chromosomes; Cyclohexanones; Disease Models, Animal; Female; Fetus; Fibroblasts; Genetic Complementation Test; Genetic Therapy; Genetic Vectors; Humans; Hydrolases; Induced Pluripotent Stem Cells; Lentivirus; Liver; Mice; Mice, Knockout; Nitrobenzoates; Pregnancy; Promoter Regions, Genetic; Spleen Focus-Forming Viruses; Tetraploidy; Tyrosinemias

2011
Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism.
    The Journal of clinical investigation, 2011, Volume: 121, Issue:10

    Topics: Albinism, Oculocutaneous; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Enzyme Stability; Eye Color; Female; Humans; Melanins; Melanocytes; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Microscopy, Electron, Transmission; Models, Molecular; Monophenol Monooxygenase; Mutation; Nitrobenzoates; Pregnancy; Skin Pigmentation; Tyrosine

2011
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
Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:3

    Topics: Animals; Animals, Newborn; Antioxidants; Ascorbic Acid; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Drug Therapy, Combination; Female; Glutathione; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Oxidative Stress; Pregnancy; Tyrosinemias

2008
Hepatic stress in hereditary tyrosinemia type 1 (HT1) activates the AKT survival pathway in the fah-/- knockout mice model.
    Journal of hepatology, 2008, Volume: 48, Issue:2

    Topics: Animals; bcl-X Protein; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Proto-Oncogene Proteins c-akt; Proto-Oncogene Proteins c-bcl-2; Signal Transduction; Tyrosinemias

2008
Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I.
    Nature genetics, 1995, Volume: 10, Issue:4

    Topics: alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Female; Heptanoates; Humans; Hydrolases; Liver; Liver Diseases; Liver Neoplasms; Male; Mice; Mice, Inbred C57BL; Nitrobenzoates; Pancreas; RNA, Messenger; Tyrosine

1995
Hepatocytes corrected by gene therapy are selected in vivo in a murine model of hereditary tyrosinaemia type I.
    Nature genetics, 1996, Volume: 12, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cell Count; Cell Transplantation; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genetic Therapy; Humans; Hydrolases; Immunoenzyme Techniques; Liver; Liver Function Tests; Liver Neoplasms; Male; Mice; Mice, Inbred C57BL; Nitrobenzoates; Retroviridae; Tyrosine

1996
Ex vivo hepatic gene therapy of a mouse model of Hereditary Tyrosinemia Type I.
    Human gene therapy, 1998, Feb-10, Volume: 9, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Base Sequence; Cell Survival; Cell Transplantation; Cells, Cultured; Cyclohexanones; Disease Models, Animal; DNA, Complementary; Enzyme Inhibitors; Female; Genetic Diseases, Inborn; Genetic Therapy; Humans; Hydrolases; Liver; Male; Mice; Molecular Sequence Data; Nitrobenzoates; Tyrosine

1998
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
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