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