Compounds > 1-deoxynojirimycin
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1-deoxynojirimycin and Eccentro-Osteochondrodysplasia

1-deoxynojirimycin has been researched along with Eccentro-Osteochondrodysplasia in 7 studies

Research

Studies (7)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (14.29)29.6817
2010's6 (85.71)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Ballhausen, D; Biela-Banaś, A; Burda, P; Caciotti, A; Charollais-Thoenig, J; Demotz, S; Front, S; Gallienne, E; Higaki, K; Martin, OR; Morrone, A1
Aguilar-Moncayo, M; Garcia Fernandez, JM; Higaki, K; Mena-Barragán, T; Nanba, E; Ohto, U; Ortiz Mellet, C; Shimizu, T; Suzuki, H; Suzuki, Y1
Fischer, R; Gonzalez Santana, A; Pabst, BM; Paschke, E; Schalli, M; Stütz, AE; Thonhofer, M; Tschernutter, M; Tysoe, C; Weber, P; Windischhofer, W; Withers, SG1
Fantur, K; Hofer, D; Pabst, BM; Paschke, E; Schitter, G; Steiner, AJ; Stütz, AE; Wrodnigg, TM1
Ochi, T; Ohto, U; Satow, Y; Shimizu, T; Usui, K; Yuki, K1
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, K1
Matsuda, J; Nanba, E; Ogawa, Y; Ohno, K; Oshima, A; Suzuki, Y; Taniguchi, M; Tominaga, L1

Other Studies

7 other study(ies) available for 1-deoxynojirimycin and Eccentro-Osteochondrodysplasia

ArticleYear
(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.
    European journal of medicinal chemistry, 2017, Jan-27, Volume: 126

    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.
    The Journal of biological chemistry, 2014, May-23, Volume: 289, Issue:21

    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.
    Carbohydrate research, 2016, Jun-24, Volume: 429

    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.
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:3

    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.
    The Journal of biological chemistry, 2012, Jan-13, Volume: 287, Issue:3

    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.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2013, Volume: 21, Issue:3

    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.
    Brain & development, 2001, Volume: 23, Issue:5

    Topics: 1-Deoxynojirimycin; Animals; beta-Galactosidase; Cells, Cultured; DNA, Complementary; Fibroblasts; Gangliosidosis, GM1; Humans; Mice; Mice, Knockout; Mucopolysaccharidosis IV; Mutation

2001