(2R,3S,4R,5S)-1-Butyl-2-(hydroxymethyl)piperidine-3,4,5-triol
| Title | Journal |
|---|---|
| α-Galactosidase aggregation is a determinant of pharmacological chaperone efficacy on Fabry disease mutants. | The Journal of biological chemistry 20120817 |
| Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease. | Journal of inherited metabolic disease 20120501 |
| Crystal structure of human β-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases. | The Journal of biological chemistry 20120113 |
| Pharmacological chaperone therapy for Fabry disease. | Proceedings of the Japan Academy. Series B, Physical and biological sciences 20120111 |
| The molecular basis of pharmacological chaperoning in human α-galactosidase. | Chemistry & biology 20111223 |
| Murine β-galactosidase stability is not dependent on temperature or protective protein/cathepsin A. | Molecular genetics and metabolism 20111201 |
| A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease. | Human mutation 20110801 |
| Treatment of fabry disease: current and emerging strategies. | Current pharmaceutical biotechnology 20110601 |
| Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease. | Journal of biochemistry 20110201 |
| Novel method for chase analysis of oligosaccharide metabolic error caused by xenobiotics. | Analytical biochemistry 20101001 |
| Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice. | Journal of neurochemistry 20100601 |
| Lysosomal storage of oligosaccharide and glycosphingolipid in imino sugar treated cells. | Glycoconjugate journal 20100401 |
| Short and straightforward synthesis of (-)-1-deoxygalactonojirimycin. | Organic letters 20100319 |
| Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation. | Journal of medicinal chemistry 20100128 |
| The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease. | Molecular therapy : the journal of the American Society of Gene Therapy 20100101 |
| Raffinose and stachyose metabolism are not required for efficient soybean seed germination. | Journal of plant physiology 20090815 |
| Synthesis and characterization of a new fluorogenic substrate for alpha-galactosidase. | Analytical and bioanalytical chemistry 20090801 |
| The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines. | Journal of inherited metabolic disease 20090601 |
| Glucosylceramide synthase inhibitors sensitise CLL cells to cytotoxic agents without reversing P-gp functional activity. | European journal of pharmacology 20090501 |
| Molecular interaction of imino sugars with human alpha-galactosidase: Insight into the mechanism of complex formation and pharmacological chaperone action in Fabry disease. | Molecular genetics and metabolism 20090401 |
| Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease. | The Journal of pharmacology and experimental therapeutics 20090301 |
| Effects of a chemical chaperone on genetic mutations in alpha-galactosidase A in Korean patients with Fabry disease. | Experimental & molecular medicine 20090131 |
| Pharmacological chaperone therapy by active-site-specific chaperones in Fabry disease: in vitro and preclinical studies. | International journal of clinical pharmacology and therapeutics 20090101 |
| Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone. | Pharmacogenetics and genomics 20080901 |
| Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis. | Molecular genetics and metabolism 20080601 |
| N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice. | Neurochemistry international 20080501 |
| Accumulation of glucosylceramide in murine testis, caused by inhibition of beta-glucosidase 2: implications for spermatogenesis. | The Journal of biological chemistry 20071109 |
| Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin. | The Biochemical journal 20070901 |
| Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. | Nature medicine 20070401 |
| Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variants. | American journal of physiology. Cell physiology 20060401 |
| Emerging strategies for the treatment of hereditary metabolic storage disorders. | Rejuvenation research 20060101 |
| Substrate reduction therapy of glycosphingolipid storage disorders. | Journal of inherited metabolic disease 20060101 |
| Alkylated imino sugars, reversible male infertility-inducing agents, do not affect the genetic integrity of male mouse germ cells during short-term treatment despite induction of sperm deformities. | Biology of reproduction 20050401 |
| Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice. | Journal of lipid research 20050401 |
| A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder. | FASEB journal : official publication of the Federation of American Societies for Experimental Biology 20050101 |
| NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin. | Annals of neurology 20041101 |
| Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease. | Neurobiology of disease 20040801 |
| Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport. | The Journal of biological chemistry 20040618 |
| Chemical chaperones--a new concept in drug research. | Chembiochem : a European journal of chemical biology 20030404 |
| Structure-activity relationship of a new class of anti-hepatitis B virus agents. | Antimicrobial agents and chemotherapy 20021201 |
| [Fabry's disease (alpha-galactosidase-A deficiency): recent therapeutic innovations]. | Journal de la Societe de biologie 20020101 |
| Galactonojirimycin derivatives restore mutant human beta-galactosidase activities expressed in fibroblasts from enzyme-deficient knockout mouse. | Brain & development 20010801 |
| Carbohydrate receptor depletion as an antimicrobial strategy for prevention of urinary tract infection. | The Journal of infectious diseases 20010301 |