N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice
- 8 December 2007
- journal article
- Published by Elsevier in Neurochemistry International
- Vol. 52 (6) , 1125-1133
- https://doi.org/10.1016/j.neuint.2007.12.001
Abstract
No abstract availableKeywords
This publication has 66 references indexed in Scilit:
- Pyrimethamine as a Potential Pharmacological Chaperone for Late-onset Forms of GM2 GangliosidosisJournal of Biological Chemistry, 2007
- High-Throughput Screening for Human Lysosomal β-N-Acetyl Hexosaminidase Inhibitors Acting as Pharmacological ChaperonesChemistry & Biology, 2007
- Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff diseaseJournal of Neurochemistry, 2004
- Ganglioside Glycosyltransferases Organize in Distinct Multienzyme Complexes in CHO-K1 CellsJournal of Biological Chemistry, 2003
- New Prospects for the Treatment of Lysosomal Storage DiseasesDrugs, 2002
- Galactonojirimycin derivatives restore mutant human β-galactosidase activities expressed in fibroblasts from enzyme-deficient knockout mouseBrain & Development, 2001
- Differential Expression of Three Sialidase Genes in Rat DevelopmentBiochemical and Biophysical Research Communications, 2001
- The Biochemical Basis of GangliosidosesNeuropediatrics, 1984
- Genetic analysis of cerebellar lipids in mice susceptible to audiogenic seizuresExperimental Neurology, 1984
- Biosynthesis of brain gangliosidesMolecular and Cellular Biochemistry, 1974