1-deoxynojirimycin and Adult Sandhoff Disease

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

Topics: 1-Deoxynojirimycin; Animals; Enzyme Inhibitors; Gaucher Disease; Genistein; Humans; Lysosomal Storage Diseases; Lysosomes; Mucopolysaccharidosis III; Niemann-Pick Diseases; Sandhoff Disease; Tay-Sachs Disease

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

Topics: 1-Deoxynojirimycin; Child; Child, Preschool; Diet, Ketogenic; Humans; Male; Sandhoff Disease

Topics: 1-Deoxynojirimycin; Adolescent; Child, Preschool; Disease Progression; Enzyme Inhibitors; Glucosyltransferases; Hexosaminidase B; Humans; Male; Mutation; 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

Topics: 1-Deoxynojirimycin; 3-Hydroxybutyric Acid; Analysis of Variance; Animals; beta-N-Acetylhexosaminidases; Blood Glucose; Body Weight; Brain; Chromatography, High Pressure Liquid; Chromatography, Thin Layer; Diet, Ketogenic; Eating; G(M2) Ganglioside; Lipid Metabolism; Mice; Mice, Knockout; Myelin Sheath; Purkinje Cells; Sandhoff Disease

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

Topics: 1-Deoxynojirimycin; Animals; beta-N-Acetylhexosaminidases; G(M2) Ganglioside; Hexosaminidase B; Mice; Neural Stem Cells; Sandhoff Disease

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

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

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

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

Topics: 1-Deoxynojirimycin; Animals; beta-N-Acetylhexosaminidases; Brain; Embryonic Stem Cells; Humans; Immunohistochemistry; Mice; Mice, Knockout; Microglia; Neurons; Patch-Clamp Techniques; Sandhoff Disease; Stem Cell Transplantation

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

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

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