miglustat has been researched along with Sandhoff Disease in 11 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 2 (18.18) | 18.2507 |
| 2000's | 7 (63.64) | 29.6817 |
| 2010's | 2 (18.18) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Hopkin, RJ; Hufnagel, SB; Jefferies, JL; Karl, G; Pabón, LA; Prada, CE; Serrano, NC; Villamizar-Schiller, IT | 1 |
| Dekomien, G; Lefeber, DJ; Morava, E; Wevers, RA; Willemsen, MA; Wortmann, SB | 1 |
| Berg, JE; Tallaksen, CM | 1 |
| Guitton, J; Masciullo, M; Modoni, A; Ricci, E; Santoro, M; Silvestri, G; Tonali, P | 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 |
| Futerman, AH; Jeyakumar, M; Lloyd-Evans, E; Pelled, D; Platt, FM; Riebeling, C | 1 |
| Jakóbkiewicz-Banecka, J; Wegrzyn, A; Wegrzyn, G | 1 |
| Chavany, C; Jendoubi, M | 1 |
| Butters, TD; Cortina-Borja, M; Dwek, RA; Hunnam, V; Jeyakumar, M; Perry, VH; Platt, FM; Proia, RL | 1 |
| Butters, TD; Cortina-Borja, M; Dwek, RA; Jeyakumar, M; Norflus, F; Perry, VH; Platt, FM; Proia, RL; Tifft, CJ | 1 |
3 review(s) available for miglustat and Sandhoff Disease
| 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 |
8 other study(ies) available for miglustat and Sandhoff Disease
| Article | Year |
|---|---|
Neurological and cardiac responses after treatment with miglustat and a ketogenic diet in a patient with Sandhoff disease.
Topics: 1-Deoxynojirimycin; Child; Child, Preschool; Diet, Ketogenic; Humans; Male; Sandhoff Disease | 2015 |
Substrate deprivation therapy in juvenile Sandhoff disease.
Topics: 1-Deoxynojirimycin; Adolescent; Child, Preschool; Disease Progression; Enzyme Inhibitors; Glucosyltransferases; Hexosaminidase B; Humans; Male; Mutation; Sandhoff Disease | 2009 |
Miglustat therapy in juvenile Sandhoff disease.
Topics: 1-Deoxynojirimycin; Adolescent; beta-Hexosaminidase beta Chain; Cerebellum; Child, Preschool; Depression; Disease Progression; Electroconvulsive Therapy; Enzyme Inhibitors; Glucosyltransferases; Humans; Male; Mutation; Sandhoff Disease | 2009 |
Substrate reduction therapy with miglustat in chronic GM2 gangliosidosis type Sandhoff: results of a 3-year follow-up.
Topics: 1-Deoxynojirimycin; Disease Progression; Enzyme Inhibitors; Glucosyltransferases; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Weakness; Muscular Atrophy, Spinal; Neurologic Examination; Predictive Value of Tests; Sandhoff Disease; Time Factors; Treatment Outcome | 2010 |
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 |
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 |
Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.
Topics: 1-Deoxynojirimycin; Aging; Animals; Apoptosis; Behavior, Animal; beta-N-Acetylhexosaminidases; Brain; Dentate Gyrus; Enzyme Inhibitors; Female; Genes, Recessive; Glycoside Hydrolase Inhibitors; Glycosphingolipids; Hexosaminidase B; Life Expectancy; Liver; Mice; Mice, Mutant Strains; Motor Activity; Sandhoff Disease | 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 |