1-deoxynojirimycin has been researched along with Disease Models, Animal in 66 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 5 (7.58) | 18.2507 |
| 2000's | 22 (33.33) | 29.6817 |
| 2010's | 37 (56.06) | 24.3611 |
| 2020's | 2 (3.03) | 2.80 |
| Authors | Studies |
|---|---|
| Antipova, V; Holzmann, C; Rolfs, A; Witt, M; Wree, A | 1 |
| Honda, Y; Imajo, K; Iwaki, M; Kessoku, T; Kobayashi, T; Nagashima, Y; Nakajima, A; Nogami, A; Ogawa, Y; Ozaki, A; Saito, S; Tomeno, W; Yoneda, M | 1 |
| Kishida, Y; Ohno, H; Oki, K; Okubo, H; Yoneda, M | 1 |
| Günther, R; Holzmann, C; Meyer, A; Rolfs, A; Schmitt, O; Witt, M; Wree, A | 1 |
| Boudewyn, LC; Dobrenis, K; Grishchuk, Y; Kuchar, L; Ledvinova, J; Sikora, J; Walkley, SU; Wang, SL | 1 |
| Chen, W; Li, C; Liang, T; Peng, G; Shen, Z; Wang, F; Wu, X; Zheng, Y; Zuo, W | 1 |
| Iwasa, M; Kanamori, H; Kawaguchi, T; Kawasaki, M; Mikami, A; Minatoguchi, S; Naruse, G; Nishigaki, K; Yamada, Y; Yoshida, A | 1 |
| Do, HV; Feng, J; Frascella, M; Garcia, A; Gotschall, R; Khanna, R; Lun, Y; Martina, JA; Nair, A; Ponery, AS; Puertollano, R; Raben, N; Ralston, E; Schilling, A; Soska, R; Tuske, S; Valenzano, KJ; Valle, MCD; Xu, S | 1 |
| Bräuer, AU; Burstein, C; Engelmann, R; Frank, M; Gläser, A; Gräler, M; Müller-Hilke, B; Neidhardt, J; Neßlauer, AM; Rolfs, A; Witt, M; Wree, A | 1 |
| Ikenoya, S; Kitatani, T; Takahashi, S | 1 |
| Blank, PS; Cluzeau, CV; Cologna, SM; Dail, MK; Lieberman, AP; Porter, FD; Siebel, S; Toth, CL; Wassif, CA; Yanjanin, NM | 1 |
| Frech, MJ; Holzmann, C; Hovakimyan, M; Hübner, R; Lukas, J; Maass, F; Petersen, J; Rolfs, A; Witt, M; Wree, A | 1 |
| Haÿ, E; Jacquot, JP; Le Henaff, C; Marie, PJ; Marty, C; Tabary, O; Velard, F | 1 |
| Brignol, N; Dhulipala, R; Do, HV; Feng, J; Frascella, M; Garcia, A; Khanna, R; Lockhart, DJ; Lun, Y; Pellegrino, LJ; Powe, AC; Soska, R; Toth, MJ; Valenzano, KJ; Wustman, BA; Xu, S | 1 |
| Agovino, T; Andria, G; Ascione, S; Danesino, C; De Filippi, P; Della Casa, R; Donati, MA; Fecarotta, S; la Marca, G; Mollica, C; Morandi, LO; Nusco, E; Ombrone, D; Parenti, G; Pasanisi, MB; Pichiecchio, A; Porto, C; Ravaglia, S; Romano, A; Rosa, M; Rossi, B; Sacchini, M | 1 |
| Hawlitschka, A; Hovakimyan, M; Lukas, J; Maass, F; Petersen, J; Rolfs, A; Schmitt, O; Witt, M; Wree, A | 1 |
| Alvarez-Fischer, D; Andreas, H; Hirsch, EC; Höglinger, GU; Höllerhage, M; Lu, L; Noelker, C; Oertel, WH; Roscher, R; Sturn, A; Vulinovic, F | 1 |
| Cardinale, A; Cutarelli, A; D'Arcangelo, G; Frank, C; Grossi, D; Merlo, D; Racaniello, M; Rufini, S; Tancredi, V; Zaratti, A | 1 |
| Alonzi, DS; Beatty, PR; Buck, MD; Caputo, AT; Dwek, RA; Eddy, W; Enterlein, SG; Harris, E; Hill, ML; Ide, D; Iwaki, R; Kato, A; Khaliq, M; Kiappes, JL; Killingbeck, SS; Kinami, K; King, K; Miller, JL; Plummer, EM; Ramstedt, U; Sampath, A; Sayce, AC; Shresta, S; Tang, W; Treston, AM; Tyrrell, BE; Warfield, KL; Zitzmann, N | 1 |
| Barnard, DL; Callahan, MV; Day, CW; Enterlein, SG; Khaliq, M; Ramstedt, U; Sampath, A; Smee, DF; Warfield, KL | 1 |
| Byers, S; Derrick-Roberts, A; Fletcher, J; Kaidonis, X; Ranieri, E; Sharp, P | 1 |
| Grittner, U; Holzmann, C; Rolfs, A; Schlegel, V; Thieme, M; Witt, M; Wree, A | 1 |
| Alonzi, DS; Bentley, EM; Dowall, SD; Dwek, RA; Hall, G; Hewson, R; Miller, JL; Rule, A; Sayce, AC; Spiro, SG; Taylor, I; Thom, R; Wright, E; Zitzmann, N | 1 |
| Agans, KN; Callahan, MV; Garza, NL; Geisbert, JB; Geisbert, TW; Klose, B; Mire, CE; Qiu, X; Ramstedt, U; Shurtleff, AC; Stuthman, KS; Treston, AM; Van Tongeren, SA; Warfield, KL; Warren, TK; Wells, J; Wong, G | 1 |
| Chang, HH; Fan, JQ; Higuchi, Y; Ishii, S; Mannen, K; Shimada, T; Taguchi, A; Yoshioka, H | 1 |
| Aerts, JM; Aten, J; Bijl, N; Boot, RG; Claessen, N; Groen, AK; Langeveld, M; Moerland, PD; Ottenhoff, R; Sokolović, M; van Eijk, M; van Roomen, CP; Vrins, C | 1 |
| Ali, NF; Davidson, CD; Dobrenis, K; Micsenyi, MC; Ory, DS; Renault, S; Stephney, G; Vanier, MT; Walkley, SU | 1 |
| Benjamin, ER; Brignol, N; Desnick, RJ; Feng, J; Frascella, M; Khanna, R; Lockhart, DJ; Lun, Y; Pellegrino, L; Sitaraman, SA; Soska, R; Valenzano, KJ; Young, B | 1 |
| Aerts, JM; Bietrix, F; Groen, AK; Lombardo, E; Ottenhoff, R; Rensen, PC; van Roomen, CP; Verhoeven, AJ; Vos, M | 1 |
| Furukawa, K; Hamanaka, R; Ishii, S; Kulkarni, AB; Kunieda, T; Mannen, K; Matsuda, J; Noguchi, Y; Shiozuka, C; Taguchi, A; Uchio-Yamada, K; Yano, S; Yokoyama, S; Yoshioka, H | 1 |
| Hughes, DA; Pastores, GM | 1 |
| Berton, G; Bezzerri, V; Cabrini, G; Cioffi, F; Dechecchi, MC; Gambari, R; Huang, S; Lampronti, I; Mazzi, P; Nicolis, E; Scupoli, MT; Wiszniewski, L | 1 |
| Cho, JK; Curtis-Long, MJ; Jung, S; Kim, YS; Lee, BW; Park, KH; Ryu, HW; Yuk, HJ | 1 |
| Aerts, JM; Aten, J; Bietrix, FM; Bijl, N; Boot, RG; Dekker, N; Groen, AK; Groener, J; Langeveld, M; Lombardo, E; Ottenhoff, R; Overkleeft, HS; Serlie, M; van Eijk, M; van Roomen, C; Wennekes, T | 1 |
| Donald, A; Oko, R; van der Spoel, AC; Xu, W | 1 |
| Fornfeist, S | 1 |
| Ishii, S | 1 |
| Benedict, D; Buttner, J; Callahan, JW; Crushell, E; Mahuran, DJ; Martin, DR; Rigat, BA; Tropak, MB | 1 |
| Feng, J; Flanagan, JJ; Frascella, M; Guillen, D; Khanna, R; Lockhart, DJ; Lun, Y; Pellegrino, LJ; Soska, R; Valenzano, KJ | 1 |
| Igdoura, SA; Venier, RE | 1 |
| Hibi, T; Miura, S; Mizushima, T; Nagata, H; Nakashita, M; Suzuki, H; Taki, T; Uehara, K | 1 |
| Aguilar-Moncayo, M; García Fernández, JM; García-Moreno, MI; Higaki, K; Hirano, Y; Mena-Barragán, T; Nanba, E; Ninomiya, H; Ohno, K; Ortiz Mellet, C; Sakakibara, Y; Suzuki, Y; Takai, T; Yu, L; Yura, K | 1 |
| Germain, DP | 1 |
| Butters, TD; Dwek, RA; Jeyakumar, M; Platt, FM | 1 |
| Futerman, AH; Jeyakumar, M; Lloyd-Evans, E; Pelled, D; Platt, FM; Riebeling, C | 1 |
| Andersson, U; Butters, TD; Dwek, RA; Heare, T; Jeyakumar, M; Platt, FM | 1 |
| Butters, TD; Dwek, RA; Platt, FM | 1 |
| D'Azzo, A; El-Abbadi, MM; Hauser, EC; Kasperzyk, JL; Platt, FM; Seyfried, TN | 1 |
| Aerts, JM; Boon, L; Bullens, D; Ceuppens, JL; Geboes, K; Kasran, A; Maerten, P; Rutgeerts, P; Shen, C; Van Assche, G | 1 |
| Andersson, U; Borja, MC; Butters, TD; Dwek, RA; Jeyakumar, M; Platt, FM; Smith, D | 1 |
| Borja, MC; Butters, TD; Dwek, RA; Jeyakumar, M; Neville, DC; Platt, FM; Smith, DA; Williams, IM | 1 |
| Fan, JQ; Ishii, S; Kulkarni, AB; Mannen, K; Yoshioka, H | 1 |
| Alp, NJ; Butters, TD; Channon, KM; Clarke, K; Dwek, RA; Heare, T; Kulkarni, AB; Platt, FM; Priestman, DA; Qasba, P | 1 |
| Flamand, M; Liu, W; Schul, W; Vasudevan, SG; Xu, HY | 1 |
| Itoh, K; Tsuji, D | 1 |
| Baek, RC; Kasperzyk, JL; Platt, FM; Seyfried, TN | 1 |
| Butters, TD; d'Azzo, A; Dwek, RA; Elliot-Smith, E; Jeyakumar, M; Lloyd-Evans, E; Platt, FM; Smith, DA; Speak, AO; van der Spoel, AC | 1 |
| Butters, TD; Dwek, RA; Karlsson, GB; Neises, GR; Platt, FM | 1 |
| Butters, TD; Dwek, RA; Neises, GR; Perry, VH; Platt, FM; Proia, RL; Reinkensmeier, G; Townsend, MJ; Winchester, B | 1 |
| Chavany, C; Jendoubi, M | 1 |
| Axen, KV; Li, X; Sclafani, A | 1 |
| Dolphin, PJ; Graham, SE; Russell, JC | 1 |
| Butters, TD; Cortina-Borja, M; Dwek, RA; Jeyakumar, M; Norflus, F; Perry, VH; Platt, FM; Proia, RL; Tifft, CJ | 1 |
| Arai, M; Fujiwara, H; Fujiwara, T; Hashimoto, K; Hashimoto, Y; Minatoguchi, S; Nishida, Y; Takemura, G; Uno, Y; Wang, N; Wu, DJ | 1 |
| Somers, KL; Thrall, MA; Walkley, SU; Zervas, M | 1 |
| Arai, M; Chen, XH; Fujiwara, H; Fujiwara, T; Hashimoto, K; Minatoguchi, S; Nishida, Y; Takemura, G; Uno, Y; Wang, N | 1 |
10 review(s) available for 1-deoxynojirimycin and Disease Models, Animal
| Article | Year |
|---|---|
[Pharmacological and clinical profiles of miglustat (Brazaves(®)) for the treatment of Niemann-Pick type C disease].
Topics: 1-Deoxynojirimycin; Animals; Calcium; Clinical Trials as Topic; Disease Models, Animal; Enzyme Inhibitors; Glucosyltransferases; Humans; Lysosomes; Mice; Niemann-Pick Disease, Type C | 2013 |
The pathophysiology of GD - current understanding and rationale for existing and emerging therapeutic approaches.
Topics: 1-Deoxynojirimycin; Animals; Autophagy; Calcium; Cell Membrane; Disease Models, Animal; DNA Mutational Analysis; Endoplasmic Reticulum; Enzyme Inhibitors; Enzyme Replacement Therapy; Gaucher Disease; Genetic Therapy; Glucosylceramidase; Glucosylceramides; Glycolipids; Homeostasis; Humans; Mice; Oxidative Stress; Proteostasis Deficiencies | 2010 |
Glycosphingolipids and insulin resistance.
Topics: 1-Deoxynojirimycin; Adamantane; Animals; Cardiovascular Diseases; Ceramides; Diabetes Mellitus, Type 2; Dioxanes; Disease Models, Animal; Fatty Acids; Fatty Liver; Gaucher Disease; Glucosyltransferases; Glycosphingolipids; Humans; Insulin Resistance; Metabolic Syndrome; Mice; Mice, Obese; Non-alcoholic Fatty Liver Disease; Obesity; Pyrrolidines; Receptor, Insulin; Signal Transduction | 2011 |
Miglustat as a therapeutic agent: prospects and caveats.
Topics: 1-Deoxynojirimycin; Animals; Clinical Trials as Topic; Disease Models, Animal; Gangliosides; Gaucher Disease; Humans | 2012 |
[Fabry's disease (alpha-galactosidase-A deficiency): recent therapeutic innovations].
Topics: 1-Deoxynojirimycin; Adenoviridae; alpha-Galactosidase; Analgesics; Animals; Antihypertensive Agents; Clinical Trials as Topic; Combined Modality Therapy; Disease Models, Animal; Enzyme Inhibitors; Fabry Disease; Genetic Therapy; Genetic Vectors; Humans; Kidney Diseases; Kidney Transplantation; Mice; Mice, Knockout; Recombinant Fusion Proteins; Renal Dialysis; Retroviridae; X Chromosome | 2002 |
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
Topics: 1-Deoxynojirimycin; Animals; Bone Marrow Transplantation; Chemotherapy, Adjuvant; Disease Models, Animal; G(M2) Ganglioside; Gangliosides; Glucosylceramides; Glucosyltransferases; Glycosphingolipids; Humans; Lysosomal Storage Diseases; Lysosomes; Mice; Models, Biological; Models, Chemical; Morpholines; Sandhoff Disease; Tay-Sachs Disease; Treatment Outcome | 2002 |
Substrate reduction therapy in mouse models of the glycosphingolipidoses.
Topics: 1-Deoxynojirimycin; Animals; Disease Models, Animal; Enzyme Inhibitors; Mice; Sphingolipidoses; Substrate Specificity | 2003 |
New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases.
Topics: 1-Deoxynojirimycin; Animals; Disease Models, Animal; Drug Therapy, Combination; Enzyme Inhibitors; Gaucher Disease; Glycosphingolipids; Humans; In Vitro Techniques; Lysosomal Storage Diseases; Mice | 2003 |
[Molecular pathogenesis and therapeutic targets of lysosomal diseases].
Topics: 1-Deoxynojirimycin; Animals; Anti-Inflammatory Agents, Non-Steroidal; Aspirin; beta-N-Acetylhexosaminidases; Bone Marrow Transplantation; Disease Models, Animal; G(M2) Activator Protein; Genetic Therapy; Humans; Lysosomal Storage Diseases, Nervous System; Mutation | 2007 |
Biology and potential strategies for the treatment of GM2 gangliosidoses.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; Animals; beta-N-Acetylhexosaminidases; Bone Marrow Transplantation; Cats; Cell Transplantation; Child; Disease Models, Animal; Dogs; G(M2) Ganglioside; Genetic Therapy; Genetic Vectors; Glycolipids; HIV; Humans; Infant; Lysosomes; Mice; Mice, Knockout; Neurons; Phenotype; Point Mutation; Rats; Sandhoff Disease; Swine; Tay-Sachs Disease; Transplantation, Homologous | 1998 |
56 other study(ies) available for 1-deoxynojirimycin and Disease Models, Animal
| Article | Year |
|---|---|
Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1.
Topics: 1-Deoxynojirimycin; 2-Hydroxypropyl-beta-cyclodextrin; Animals; Cyclodextrins; Disease Models, Animal; Drug Therapy, Combination; Female; Male; Mice; Mice, Inbred BALB C; Niemann-Pick Disease, Type C; Pregnanolone | 2021 |
Protective effect of SGL5213, a potent intestinal sodium-glucose cotransporter 1 inhibitor, in nonalcoholic fatty liver disease in mice.
Topics: 1-Deoxynojirimycin; Animals; Chronic Disease; Diet, High-Fat; Dietary Sucrose; Disease Models, Animal; Gastrointestinal Absorption; Gene Expression; Glucose; Insulin Resistance; Male; Mice, Inbred C57BL; Non-alcoholic Fatty Liver Disease; Obesity; Patient Acuity; RNA, Messenger; Sodium-Glucose Transporter 1; Sorbitol | 2021 |
Effect of miglitol on the suppression of nonalcoholic steatohepatitis development and improvement of the gut environment in a rodent model.
Topics: 1-Deoxynojirimycin; Animals; Diet, High-Fat; Disease Models, Animal; Gastrointestinal Microbiome; Glucagon-Like Peptide 1; Glycoside Hydrolase Inhibitors; Insulin Resistance; Male; Mice; Mice, Inbred C57BL; Non-alcoholic Fatty Liver Disease | 2017 |
Increased Regenerative Capacity of the Olfactory Epithelium in Niemann-Pick Disease Type C1.
Topics: 1-Deoxynojirimycin; 2-Hydroxypropyl-beta-cyclodextrin; Animals; Apoptosis; beta-Cyclodextrins; Cell Proliferation; Disease Models, Animal; Humans; Intracellular Signaling Peptides and Proteins; Mice; Mutation; Niemann-Pick C1 Protein; Niemann-Pick Disease, Type C; Olfactory Mucosa; Pregnanolone; Proteins | 2017 |
N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.
Topics: 1-Deoxynojirimycin; Animals; Antigens, CD; Cell Count; Cerebellum; Disease Models, Animal; Enzyme Inhibitors; Exploratory Behavior; Gliosis; Lipid Metabolism; Mice; Mice, Inbred C57BL; Mice, Transgenic; Movement Disorders; Mucolipidoses; Nerve Tissue Proteins; Psychomotor Performance; Purkinje Cells; Retina; Transient Receptor Potential Channels | 2017 |
Neuroprotective effect of 1-Deoxynojirimycin on cognitive impairment, β-amyloid deposition, and neuroinflammation in the SAMP8 mice.
Topics: 1-Deoxynojirimycin; Amyloid beta-Peptides; Amyloid Precursor Protein Secretases; Animals; Aspartic Acid Endopeptidases; Behavior, Animal; Brain-Derived Neurotrophic Factor; Cognition; Cognitive Dysfunction; Disease Models, Animal; Encephalitis; Hippocampus; Inflammation Mediators; Male; Membrane Glycoproteins; Memory; Mice, Inbred Strains; Microglia; Neuroprotective Agents; Protein-Tyrosine Kinases; Signal Transduction; Time Factors | 2018 |
The intestine responds to heart failure by enhanced mitochondrial fusion through glucagon-like peptide-1 signalling.
Topics: 1-Deoxynojirimycin; Animals; Cells, Cultured; Cyclic AMP-Dependent Protein Kinases; Disease Models, Animal; Dynamins; Enteroendocrine Cells; Glucagon-Like Peptide 1; Glucagon-Like Peptide-1 Receptor; Glycoside Hydrolase Inhibitors; GTP Phosphohydrolases; Heart Failure; Ileum; Incretins; Male; Membrane Proteins; Mitochondria, Heart; Mitochondrial Dynamics; Mitochondrial Proteins; Myocytes, Cardiac; Paracrine Communication; Peptide Fragments; Rats, Inbred Dahl; Rats, Sprague-Dawley; Signal Transduction; Sodium Chloride, Dietary; Ventricular Function, Left | 2019 |
Improved efficacy of a next-generation ERT in murine Pompe disease.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Animals; Disease Models, Animal; Enzyme Replacement Therapy; Female; Glycogen; Glycogen Storage Disease Type II; Humans; Lysosomes; Male; Mannosephosphates; Mice; Mice, Knockout; Muscle, Skeletal; Rats; Rats, Sprague-Dawley | 2019 |
A therapy with miglustat, 2-hydroxypropyl-ß-cyclodextrin and allopregnanolone restores splenic cholesterol homeostasis in Niemann-pick disease type C1.
Topics: 1-Deoxynojirimycin; 2-Hydroxypropyl-beta-cyclodextrin; Animals; Cell Separation; Disease Models, Animal; Flow Cytometry; Genotype; Lipid Metabolism; Mice; Niemann-Pick Disease, Type C; Pregnanolone; Spleen | 2019 |
Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1.
Topics: 1-Deoxynojirimycin; Adolescent; Alleles; Animals; Biomarkers; Brain; Cerebellum; Cerebral Cortex; Chemokine CCL3; Chemokine CXCL5; Child; Child, Preschool; Complement C3; Disease Models, Animal; Female; Gene Expression Regulation; Humans; Infant; Infant, Newborn; Inflammation; Interleukins; Male; Mice; Mice, Inbred BALB C; Mice, Transgenic; Niemann-Pick Disease, Type C; Young Adult | 2014 |
Combined therapy with cyclodextrin/allopregnanolone and miglustat improves motor but not cognitive functions in Niemann-Pick Type C1 mice.
Topics: 1-Deoxynojirimycin; Animals; Cognition; Cyclodextrins; Disease Models, Animal; Drug Therapy, Combination; Mice; Mice, Knockout; Microscopy, Electron, Transmission; Motor Activity; Neuroprotective Agents; Niemann-Pick Disease, Type C; Pregnanolone | 2013 |
Enhanced F508del-CFTR channel activity ameliorates bone pathology in murine cystic fibrosis.
Topics: 1-Deoxynojirimycin; Animals; Bone and Bones; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Enzyme Inhibitors; Male; Mice, Inbred CFTR; Mutation; Osteoblasts | 2014 |
The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease.
Topics: 1-Deoxynojirimycin; Administration, Oral; Animals; Biocatalysis; Biological Availability; Chlorocebus aethiops; COS Cells; Disease Models, Animal; Endoplasmic Reticulum; Enzyme Stability; Gene Knockout Techniques; Glucan 1,4-alpha-Glucosidase; Glycogen; Glycogen Storage Disease Type II; Humans; Isoenzymes; Lysosomes; Mice; Mice, Transgenic; Mutant Proteins; Mutation; Protein Transport; Proteolysis | 2014 |
A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; alpha-Glucosidases; Animals; Child; Child, Preschool; Disease Models, Animal; Dried Blood Spot Testing; Drug Synergism; Enzyme Replacement Therapy; Enzyme Stability; Female; Glycogen Storage Disease Type II; Glycoside Hydrolase Inhibitors; Humans; Male; Mice; Middle Aged; Young Adult | 2014 |
Reduced cerebellar neurodegeneration after combined therapy with cyclodextrin/allopregnanolone and miglustat in NPC1: a mouse model of Niemann-Pick type C1 disease.
Topics: 1-Deoxynojirimycin; Animals; Cell Count; Cerebellum; Cyclodextrins; Disease Models, Animal; Disease Progression; Drug Therapy, Combination; Enzyme Inhibitors; Mice; Nerve Degeneration; Neurons; Niemann-Pick Disease, Type C; Pregnanolone | 2015 |
Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease.
Topics: 1-Deoxynojirimycin; 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; Animals; Brain; Cell Count; Cells, Cultured; Disease Models, Animal; Embryo, Mammalian; Gaucher Disease; Glycoside Hydrolase Inhibitors; Histocompatibility Antigens; Inositol; L-Lactate Dehydrogenase; Male; Mesencephalon; Mice; Mice, Inbred C57BL; Mitochondrial Diseases; Neurons; Parkinsonian Disorders; Tyrosine 3-Monooxygenase | 2015 |
Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease.
Topics: 1-Deoxynojirimycin; Animals; Disease Models, Animal; Enzyme Inhibitors; Extracellular Signal-Regulated MAP Kinases; Hippocampus; Mice; Neuronal Plasticity; Niemann-Pick Disease, Type C; Phosphorylation; Synapses | 2016 |
Inhibition of endoplasmic reticulum glucosidases is required for in vitro and in vivo dengue antiviral activity by the iminosugar UV-4.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Animals; Antibodies, Viral; Antibody-Dependent Enhancement; Antiviral Agents; Cells, Cultured; Chlorocebus aethiops; Clinical Trials as Topic; Dengue Virus; Disease Models, Animal; Drugs, Investigational; Endoplasmic Reticulum; Glycoside Hydrolase Inhibitors; Humans; Inhibitory Concentration 50; Mice; Monocytes; Receptors, Interferon; Serogroup; Severe Dengue; Vero Cells | 2016 |
The Iminosugar UV-4 is a Broad Inhibitor of Influenza A and B Viruses ex Vivo and in Mice.
Topics: 1-Deoxynojirimycin; Animals; Antiviral Agents; Body Weight; Cells, Cultured; Disease Models, Animal; Epithelial Cells; Humans; Influenza A virus; Influenza B virus; Mice; Orthomyxoviridae Infections; Survival Analysis; Treatment Outcome | 2016 |
N-butyldeoxynojirimycin treatment restores the innate fear response and improves learning in mucopolysaccharidosis IIIA mice.
Topics: 1-Deoxynojirimycin; Analysis of Variance; Animals; Brain; Chemokine CCL3; Disease Models, Animal; Fear; Gangliosides; Glucosyltransferases; Glycoside Hydrolase Inhibitors; Interleukin-1beta; Maze Learning; Mice; Mucopolysaccharidosis III | 2016 |
Pharmacologic Treatment Assigned for Niemann Pick Type C1 Disease Partly Changes Behavioral Traits in Wild-Type Mice.
Topics: 1-Deoxynojirimycin; Animals; Behavior, Animal; Body Weight; Brain; Cognition; Cyclodextrins; Disease Models, Animal; Drug Administration Schedule; Drug Combinations; Hypolipidemic Agents; Male; Maze Learning; Mice; Mice, Inbred BALB C; Niemann-Pick Disease, Type C; Organ Size; Pregnanolone | 2016 |
Minimal In Vivo Efficacy of Iminosugars in a Lethal Ebola Virus Guinea Pig Model.
Topics: 1-Deoxynojirimycin; Animals; Disease Models, Animal; Ebolavirus; Guinea Pigs; Hemorrhagic Fever, Ebola; Pilot Projects | 2016 |
Assessment of the potential for host-targeted iminosugars UV-4 and UV-5 activity against filovirus infections in vitro and in vivo.
Topics: 1-Deoxynojirimycin; Animals; Antiviral Agents; Chlorocebus aethiops; Disease Models, Animal; Dose-Response Relationship, Drug; Ebolavirus; Imino Sugars; Macaca; Marburgvirus; Mice; Models, Animal; Vero Cells | 2017 |
Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Disease Models, Animal; Enzyme Inhibitors; Fabry Disease; Glycosphingolipids; Humans; Kinetics; Mice; Mice, Transgenic; Models, Molecular; Protein Folding; Tissue Distribution | 2009 |
Modulation of glycosphingolipid metabolism significantly improves hepatic insulin sensitivity and reverses hepatic steatosis in mice.
Topics: 1-Deoxynojirimycin; Adamantane; Animals; Disease Models, Animal; Fatty Liver; Glucose; Glycosphingolipids; Homeostasis; Insulin; Insulin Resistance; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Obese; Obesity; Triglycerides | 2009 |
Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression.
Topics: 1-Deoxynojirimycin; 2-Hydroxypropyl-beta-cyclodextrin; Animals; beta-Cyclodextrins; Cholesterol; Cyclodextrins; Disease Models, Animal; Disease Progression; Drug Synergism; Enzyme Inhibitors; Glycosphingolipids; Mice; Mice, Transgenic; Neurons; Niemann-Pick Disease, Type C; Pregnanolone; Treatment Outcome | 2009 |
The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Blotting, Western; Disease Models, Animal; Fabry Disease; Humans; Immunohistochemistry; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Trihexosylceramides | 2010 |
Inhibition of glycosphingolipid synthesis induces a profound reduction of plasma cholesterol and inhibits atherosclerosis development in APOE*3 Leiden and low-density lipoprotein receptor-/- mice.
Topics: 1-Deoxynojirimycin; Adamantane; Animals; Apolipoproteins E; Atherosclerosis; Bile; Cholesterol; Disease Models, Animal; Dose-Response Relationship, Drug; Down-Regulation; Enzyme Inhibitors; Feces; Female; Glucosyltransferases; Glycosphingolipids; Hyperlipidemias; Kinetics; Liver; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Mutation; Receptors, LDL | 2010 |
Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Crosses, Genetic; Disease Models, Animal; Enzyme Activation; Fabry Disease; Female; Galactosyltransferases; Humans; Kidney; Liver; Mice; Mice, Knockout; Mice, Transgenic; Molecular Chaperones; Spleen; Trihexosylceramides; Up-Regulation | 2011 |
Modulators of sphingolipid metabolism reduce lung inflammation.
Topics: 1-Deoxynojirimycin; Amitriptyline; Animals; Anti-Inflammatory Agents; Cell Line; Ceramides; Disease Models, Animal; Epithelial Cells; Gene Expression Regulation; Host-Pathogen Interactions; Humans; Immunity, Innate; Inflammation Mediators; Interleukin-8; Lipopolysaccharides; Mice; Mice, Inbred C57BL; Neutrophil Infiltration; Pneumonia; Pseudomonas aeruginosa; Respiratory Mucosa | 2011 |
α-Glucosidase inhibition and antihyperglycemic activity of prenylated xanthones from Garcinia mangostana.
Topics: 1-Deoxynojirimycin; Animals; Blood Glucose; Disease Models, Animal; Enzyme Inhibitors; Fruit; Garcinia mangostana; Glycoside Hydrolase Inhibitors; Hyperglycemia; Hypoglycemic Agents; Inhibitory Concentration 50; Male; Maltose; Phytotherapy; Plant Extracts; Postprandial Period; Rats; Rats, Sprague-Dawley; Seeds; Xanthones | 2011 |
Fusion failure of dense-cored proacrosomal vesicles in an inducible mouse model of male infertility.
Topics: 1-Deoxynojirimycin; Acrosome; Animals; Asthenozoospermia; Cell Nucleus; Disease Models, Animal; Enzyme Inhibitors; Golgi Apparatus; Male; Membrane Fusion; Mice; Secretory Vesicles; Spermatids; Spermatogenesis; Ultrasonography | 2011 |
[Report of attendance at the Parseghian Scientific Conference for Niemann-Pick Type C (NP-C) Research, South Bend, Indiana: in search of new therapeutic possibilities for a very rare illness].
Topics: 1-Deoxynojirimycin; 2-Hydroxypropyl-beta-cyclodextrin; Adolescent; Animals; beta-Cyclodextrins; Child; Disease Models, Animal; Diseases in Twins; Drugs, Investigational; Histone Deacetylases; Humans; Infusions, Intravenous; Injections, Spinal; Mice; Niemann-Pick Disease, Type C; Research; United States; United States Food and Drug Administration | 2011 |
Pharmacological chaperone therapy for Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Clinical Trials as Topic; Disease Models, Animal; Fabry Disease; Female; Humans; Immunohistochemistry; Male; Mice; Mutant Proteins; Mutation; Protein Folding | 2012 |
Evaluation of N-nonyl-deoxygalactonojirimycin as a pharmacological chaperone for human GM1 gangliosidosis leads to identification of a feline model suitable for testing enzyme enhancement therapy.
Topics: 1-Deoxynojirimycin; Animals; beta-Galactosidase; Cats; Cell Line, Tumor; Disease Models, Animal; Enzyme Replacement Therapy; Fibroblasts; Gangliosidosis, GM1; Hot Temperature; Humans; Hydrogen-Ion Concentration; Mutant Proteins; Mutation; Protein Denaturation; Treatment Outcome | 2012 |
The pharmacological chaperone AT2220 increases recombinant human acid α-glucosidase uptake and glycogen reduction in a mouse model of Pompe disease.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Animals; Buffers; Disease Models, Animal; Enzyme Activation; Enzyme Stability; Glycogen; Glycogen Storage Disease Type II; Half-Life; Humans; Mice; Mice, Knockout; Protein Denaturation; Rats; Recombinant Proteins | 2012 |
Attenuation of acetic acid-induced gastric ulcer formation in rats by glucosylceramide synthase inhibitors.
Topics: 1-Deoxynojirimycin; Acetic Acid; Animals; Apoptosis; Disease Models, Animal; Enzyme Inhibitors; Fumonisins; G(M3) Ganglioside; Gastric Mucosa; Glucosylceramides; Glucosyltransferases; Humans; Male; Morpholines; Rats; Rats, Sprague-Dawley; Sphingolipids; Stomach Ulcer | 2013 |
A bicyclic 1-deoxygalactonojirimycin derivative as a novel pharmacological chaperone for GM1 gangliosidosis.
Topics: 1-Deoxynojirimycin; Administration, Oral; Animals; beta-Galactosidase; Bridged Bicyclo Compounds, Heterocyclic; Cells, Cultured; Computational Biology; Disease Models, Animal; Enzyme Inhibitors; Fibroblasts; Gangliosidosis, GM1; Imino Sugars; Lysosomes; Mice; Mice, Inbred C57BL; Mice, Knockout; Molecular Chaperones; Mucopolysaccharidosis IV; Mutation; Recombination, Genetic | 2013 |
Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.
Topics: 1-Deoxynojirimycin; Adenosine Triphosphate; Animals; Blotting, Western; Brain; Calcium; Calcium-Transporting ATPases; Cell Death; Disease Models, Animal; Dose-Response Relationship, Drug; Electrophoresis, Polyacrylamide Gel; Endoplasmic Reticulum; Enzyme Inhibitors; G(M2) Ganglioside; Gangliosides; Genotype; Glycolipids; Hippocampus; Kinetics; Lipid Metabolism; Mice; Mice, Transgenic; Microsomes; Neurons; Reverse Transcriptase Polymerase Chain Reaction; Sandhoff Disease; Sarcoplasmic Reticulum; Sarcoplasmic Reticulum Calcium-Transporting ATPases; Spectrophotometry; Thapsigargin; Time Factors | 2003 |
N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis.
Topics: 1-Deoxynojirimycin; Animals; Animals, Newborn; Body Weight; Brain; Brain Chemistry; Chromatography, Thin Layer; Disease Models, Animal; Fetal Viability; Gangliosides; Gangliosidosis, GM1; Lipid Metabolism; Lipids; Mice; Mice, Inbred C57BL; Mice, Knockout; Treatment Outcome | 2004 |
Inhibition of glycolipid biosynthesis by N-(5-adamantane-1-yl-methoxy-pentyl)-deoxynojirimycin protects against the inflammatory response in hapten-induced colitis.
Topics: 1-Deoxynojirimycin; Adamantane; Animals; Anti-Inflammatory Agents; Antibodies; Body Weight; Colitis; Colon; Disease Models, Animal; Enzyme Inhibitors; Glycolipids; Haptens; Immunoglobulin G; Immunosuppressive Agents; Interferon-gamma; Interleukin-18; Male; Mice; Mice, Inbred C57BL; Oxazolone; Peroxidase; Trinitrobenzenesulfonic Acid | 2004 |
Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease.
Topics: 1-Deoxynojirimycin; Animals; Behavior, Animal; beta-N-Acetylhexosaminidases; Body Weight; Brain; Disease Models, Animal; Female; Gangliosides; Life Expectancy; Liver; Male; Mice; Mice, Mutant Strains; Sandhoff Disease | 2004 |
NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin.
Topics: 1-Deoxynojirimycin; Age Factors; Animals; Anti-Inflammatory Agents, Non-Steroidal; Anti-Obesity Agents; Behavior, Animal; Blotting, Western; Brain; Cyclooxygenase 2; Dinoprostone; Disease Models, Animal; Drug Synergism; Enzyme Inhibitors; Enzyme-Linked Immunosorbent Assay; Glutathione; Histocompatibility Antigens Class II; Immunohistochemistry; Isoenzymes; Lipid Peroxidation; Mice; Nitric Oxide Synthase; Nitric Oxide Synthase Type II; Prostaglandin-Endoperoxide Synthases; Psychomotor Performance; Sandhoff Disease; Spinal Cord; Survival Rate; Time Factors; Vitamin A | 2004 |
Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Binding Sites; Cells, Cultured; Disease Models, Animal; Fabry Disease; Fibroblasts; Genetic Therapy; Heart; Humans; Mice; Mice, Knockout; Mice, Transgenic; Molecular Chaperones; Mutation; Myocardium; Protein Folding; RNA, Messenger; Sensitivity and Specificity; Trihexosylceramides | 2004 |
Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Animals; Aorta; Disease Models, Animal; Enzyme Inhibitors; Fabry Disease; Female; Humans; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Microscopy, Electron; Phenotype | 2007 |
A dengue fever viremia model in mice shows reduction in viral replication and suppression of the inflammatory response after treatment with antiviral drugs.
Topics: 1-Deoxynojirimycin; Animals; Antiviral Agents; Dengue; Disease Models, Animal; Dose-Response Relationship, Drug; Indolizines; Mice; Ribavirin; Time Factors; Tubercidin; Viremia; Virus Replication | 2007 |
N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice.
Topics: 1-Deoxynojirimycin; Animals; Animals, Newborn; beta-Hexosaminidase alpha Chain; Brain; Brain Chemistry; Disease Models, Animal; Down-Regulation; G(M2) Ganglioside; Gangliosides; Glycosphingolipids; Mice; Mice, Knockout; Nerve Degeneration; Neuraminidase; Sandhoff Disease; Treatment Outcome | 2008 |
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis.
Topics: 1-Deoxynojirimycin; Animals; beta-Galactosidase; Brain; Disease Models, Animal; Enzyme Inhibitors; Feces; Gangliosidosis, GM1; Glucosyltransferases; Glycosphingolipids; Humans; Macrophage Activation; Mice; Mice, Knockout; Motor Activity; Protein Transport | 2008 |
N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing.
Topics: 1-Deoxynojirimycin; alpha-Glucosidases; Animals; Cells, Cultured; Disease Models, Animal; Gaucher Disease; Glucosylceramidase; Glucosyltransferases; Glycolipids; Humans; Mice; Oligosaccharides; Structure-Activity Relationship | 1994 |
Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin.
Topics: 1-Deoxynojirimycin; Animals; Blood-Brain Barrier; Brain; Disease Models, Animal; Enzyme Inhibitors; G(M2) Ganglioside; Lysosomes; Mice; Microscopy, Electron; Neurons; Tay-Sachs Disease | 1997 |
Miglitol (BAY m 1099) treatment of diabetic hypothalamic-dietary obese rats improves islet response to glucose.
Topics: 1-Deoxynojirimycin; Animal Feed; Animals; Blood Glucose; Diabetes Mellitus; Diabetes Mellitus, Type 2; Disease Models, Animal; Female; Glucosamine; Glucose; Glycoside Hydrolase Inhibitors; Hypoglycemic Agents; Imino Pyranoses; Insulin; Insulin Secretion; Islets of Langerhans; Obesity; Rats; Rats, Sprague-Dawley; Ventromedial Hypothalamic Nucleus | 1999 |
Glucose tolerance and insulin resistance in the JCR:LA-corpulent rat: effect of miglitol (Bay m1099).
Topics: 1-Deoxynojirimycin; Animals; Blood Glucose; Body Weight; Disease Models, Animal; Eating; Enzyme Inhibitors; Glucosamine; Glucose Tolerance Test; Glycoside Hydrolase Inhibitors; Hypoglycemic Agents; Imino Pyranoses; Insulin; Insulin Resistance; Lipids; Male; Obesity; Postprandial Period; Rats | 1999 |
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.
Topics: 1-Deoxynojirimycin; Animals; beta-N-Acetylhexosaminidases; Bone Marrow Transplantation; Brain; Diagnostic Techniques, Neurological; Disease Models, Animal; Enzyme Inhibitors; Glycosphingolipids; Hexosaminidase B; Mice; Sandhoff Disease; Spinal Cord; Survival Rate | 2001 |
Combination of N-methyl-1-deoxynojirimycin and ischemic preconditioning markedly reduces the size of myocardial infarcts in rabbits.
Topics: 1-Deoxynojirimycin; Animals; Combined Modality Therapy; Disease Models, Animal; Enzyme Inhibitors; Glycogen; Glycoside Hydrolase Inhibitors; Heart Ventricles; Ischemia; Ischemic Preconditioning, Myocardial; Lactic Acid; Myocardial Infarction; Rabbits | 2001 |
Critical role for glycosphingolipids in Niemann-Pick disease type C.
Topics: 1-Deoxynojirimycin; Age Factors; Animals; Carrier Proteins; Cats; Cerebellum; Disease Models, Animal; Enzyme Inhibitors; Glycoside Hydrolase Inhibitors; Glycosphingolipids; Humans; Immunohistochemistry; Intracellular Signaling Peptides and Proteins; Membrane Glycoproteins; Mice; Niemann-Pick C1 Protein; Niemann-Pick Diseases | 2001 |
N-methyl-1-deoxynojirimycin (MOR-14), an alpha-glucosidase inhibitor, markedly improves postischemic left ventricular dysfunction.
Topics: 1-Deoxynojirimycin; Adenosine Triphosphate; Animals; Blood Flow Velocity; Disease Models, Animal; Enzyme Inhibitors; Glycogen; Glycoside Hydrolase Inhibitors; Heart; Lactic Acid; Male; Myocardial Ischemia; Myocardial Reperfusion; Rats; Rats, Sprague-Dawley; Time Factors; Ventricular Dysfunction, Left | 2000 |