1-deoxynojirimycin has been researched along with Mucopolysaccharidosis IV in 7 studies
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 1 (14.29) | 29.6817 |
2010's | 6 (85.71) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
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Ballhausen, D; Biela-Banaś, A; Burda, P; Caciotti, A; Charollais-Thoenig, J; Demotz, S; Front, S; Gallienne, E; Higaki, K; Martin, OR; Morrone, A | 1 |
Aguilar-Moncayo, M; Garcia Fernandez, JM; Higaki, K; Mena-Barragán, T; Nanba, E; Ohto, U; Ortiz Mellet, C; Shimizu, T; Suzuki, H; Suzuki, Y | 1 |
Fischer, R; Gonzalez Santana, A; Pabst, BM; Paschke, E; Schalli, M; Stütz, AE; Thonhofer, M; Tschernutter, M; Tysoe, C; Weber, P; Windischhofer, W; Withers, SG | 1 |
Fantur, K; Hofer, D; Pabst, BM; Paschke, E; Schitter, G; Steiner, AJ; Stütz, AE; Wrodnigg, TM | 1 |
Ochi, T; Ohto, U; Satow, Y; Shimizu, T; Usui, K; Yuki, 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 |
Matsuda, J; Nanba, E; Ogawa, Y; Ohno, K; Oshima, A; Suzuki, Y; Taniguchi, M; Tominaga, L | 1 |
7 other study(ies) available for 1-deoxynojirimycin and Mucopolysaccharidosis IV
Article | Year |
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(5aR)-5a-C-Pentyl-4-epi-isofagomine: A powerful inhibitor of lysosomal β-galactosidase and a remarkable chaperone for mutations associated with GM1-gangliosidosis and Morquio disease type B.
Topics: beta-Galactosidase; Drug Design; Enzyme Inhibitors; Fibroblasts; Gangliosidosis, GM1; Hot Temperature; Humans; Hydrogen-Ion Concentration; Imino Pyranoses; Lysosomes; Mucopolysaccharidosis IV; Mutation; Protein Denaturation | 2017 |
Structural basis of pharmacological chaperoning for human β-galactosidase.
Topics: 1-Deoxynojirimycin; beta-Galactosidase; Catalytic Domain; Crystallography, X-Ray; Cyclohexenes; Enzyme Inhibitors; Enzyme Stability; Gangliosidosis, GM1; Hexosamines; Humans; Hydrogen Bonding; Hydrogen-Ion Concentration; Imino Sugars; Inositol; Kinetics; Models, Molecular; Molecular Structure; Mucopolysaccharidosis IV; Mutation; Protein Structure, Tertiary; Static Electricity; Structure-Activity Relationship | 2014 |
Synthesis of C-5a-substituted derivatives of 4-epi-isofagomine: notable β-galactosidase inhibitors and activity promotors of GM1-gangliosidosis related human lysosomal β-galactosidase mutant R201C.
Topics: 1-Deoxynojirimycin; beta-Galactosidase; Gangliosidosis, GM1; Humans; Hydrophobic and Hydrophilic Interactions; Imino Pyranoses; Mucopolysaccharidosis IV | 2016 |
DLHex-DGJ, a novel derivative of 1-deoxygalactonojirimycin with pharmacological chaperone activity in human G(M1)-gangliosidosis fibroblasts.
Topics: 1-Deoxynojirimycin; Alleles; beta-Galactosidase; Cell Line; Enzyme Inhibitors; Fibroblasts; Gangliosidosis, GM1; Humans; Molecular Chaperones; Mucopolysaccharidosis IV; Mutation | 2010 |
Crystal structure of human β-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases.
Topics: 1-Deoxynojirimycin; beta-Galactosidase; Crystallography, X-Ray; G(M1) Ganglioside; Galactose; Gangliosidosis, GM1; Humans; Hydrolysis; Keratan Sulfate; Lysosomes; Models, Molecular; Mucopolysaccharidosis IV; Mutation; Protein Structure, Tertiary | 2012 |
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 |
Galactonojirimycin derivatives restore mutant human beta-galactosidase activities expressed in fibroblasts from enzyme-deficient knockout mouse.
Topics: 1-Deoxynojirimycin; Animals; beta-Galactosidase; Cells, Cultured; DNA, Complementary; Fibroblasts; Gangliosidosis, GM1; Humans; Mice; Mice, Knockout; Mucopolysaccharidosis IV; Mutation | 2001 |