miglustat has been researched along with Amaurotic Familial Idiocy in 9 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 2 (22.22) | 18.2507 |
| 2000's | 6 (66.67) | 29.6817 |
| 2010's | 1 (11.11) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Cardarelli, RA; Chen, Y; Gaval-Cruz, M; Higurashi, N; Hirose, S; Horiuchi, Y; Ishizuka, K; Kai, M; Kano, S; Kato, TA; Koga, M; Kondo, MA; Lee, Y; Maegawa, G; O'Donnell, P; Obie, C; Okano, H; Rapoport, JL; Sawa, A; Sedlak, TW; Seshadri, S; Srivastava, R; Tristan, C; Valle, D; Wilson, AM; Yuan, M | 1 |
| Gianutsos, J; Kolodny, EH; Luzy, C; Pastores, GM; Shapiro, BE | 1 |
| Butters, TD; Dwek, RA; Jeyakumar, M; Platt, FM | 1 |
| Butters, TD; d'Azzo, A; Dwek, RA; Elliot-Smith, E; Jeyakumar, M; Perry, VH; Platt, FM; Smith, DA; Thomas, R; van der Spoel, AC | 1 |
| Groot-Loonen, JJ; Hoogerbrugge, PM; Jacobs, JF; Wevers, RA; Willemsen, MA | 1 |
| Addobbati, R; Barone, R; Bembi, B; Butters, T; Cariati, R; Ciana, G; Fernandez-Guillen, L; Grasso, D; Guerci, VI; Marchetti, F; Pittis, MG | 1 |
| Jakóbkiewicz-Banecka, J; Wegrzyn, A; Wegrzyn, G | 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 |
3 review(s) available for miglustat and Amaurotic Familial Idiocy
| Article | Year |
|---|---|
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 deprivation therapy: a new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases.
Topics: 1-Deoxynojirimycin; Animals; Enzyme Inhibitors; Gaucher Disease; Genistein; Humans; Lysosomal Storage Diseases; Lysosomes; Mucopolysaccharidosis III; Niemann-Pick Diseases; Sandhoff Disease; Tay-Sachs Disease | 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 |
1 trial(s) available for miglustat and Amaurotic Familial Idiocy
| Article | Year |
|---|---|
Miglustat in late-onset Tay-Sachs disease: a 12-month, randomized, controlled clinical study with 24 months of extended treatment.
Topics: 1-Deoxynojirimycin; Adolescent; Adult; Age of Onset; Diarrhea; Enzyme Inhibitors; Fatigue; Female; Glycoside Hydrolase Inhibitors; Humans; Male; Middle Aged; Tay-Sachs Disease; Time Factors; Treatment Outcome; Weight Loss; Young Adult | 2009 |
5 other study(ies) available for miglustat and Amaurotic Familial Idiocy
| Article | Year |
|---|---|
Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathy.
Topics: 1-Deoxynojirimycin; Action Potentials; Basic Helix-Loop-Helix Transcription Factors; Cell Differentiation; Epilepsies, Myoclonic; Fibroblasts; G(M2) Ganglioside; Gene Expression; Genetic Vectors; Glycoside Hydrolase Inhibitors; Humans; Lentivirus; Nerve Tissue Proteins; Neurons; Plasmids; Primary Cell Culture; Tay-Sachs Disease; Transcription Factors; Transduction, Genetic; Transgenes | 2015 |
Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis.
Topics: 1-Deoxynojirimycin; Animals; Antigens, CD; Apoptosis; Biomarkers; Blood-Brain Barrier; Cytokines; Enzyme Inhibitors; Gangliosidoses; Gangliosidoses, GM2; Gangliosidosis, GM1; Genes, MHC Class II; Immunohistochemistry; Inflammation; Mice; Sandhoff Disease; Tay-Sachs Disease | 2003 |
Allogeneic BMT followed by substrate reduction therapy in a child with subacute Tay-Sachs disease.
Topics: 1-Deoxynojirimycin; Bone Marrow Transplantation; Child; Female; Humans; Nervous System Diseases; Tay-Sachs Disease; Transplantation, Homologous | 2005 |
Substrate reduction therapy in the infantile form of Tay-Sachs disease.
Topics: 1-Deoxynojirimycin; Craniofacial Abnormalities; Electroencephalography; Evoked Potentials, Auditory, Brain Stem; Evoked Potentials, Visual; Female; Humans; Infant; Nerve Degeneration; Tay-Sachs Disease | 2006 |
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 |