Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses von Butters, Terry D, Dwek, Raymond A, Platt, Frances M

Volltext

Targeting glycosylation as a therapeutic approach von Dwek, Raymond A, Butters, Terry D, Platt, Frances M, Zitzmann, Nicole

Volltext

Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase von Crosby, Andrew H, Simpson, Michael A, Cross, Harold, Proukakis, Christos, Priestman, David A, Neville, David C A, Reinkensmeier, Gabriele, Wang, Heng, Wiznitzer, Max, Gurtz, Kay, Verganelaki, Argyro, Pryde, Anna, Patton, Michael A, Dwek, Raymond A, Butters, Terry D, Platt, Frances M

Volltext

Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis von Jeyakumar, M., Thomas, R., Elliot‐Smith, E., Smith, D. A., van der Spoel, A. C., d’Azzo, A., Hugh Perry, V., Butters, T. D., Dwek, R. A., Platt, F. M.

Volltext

Inhibition of Glycosphingolipid Biosynthesis:  Application to Lysosomal Storage Disorders von Butters, Terry D, Dwek, Raymond A, Platt, Frances M

Volltext

Sustained therapeutic effects of oral miglustat (Zavesca, N‐butyldeoxynojirimycin, OGT 918) in type I Gaucher disease von Elstein, D., Hollak, C., Aerts, J. M. F. G., Weely, S., Maas, M., Cox, T. M., Lachmann, R. H., Hrebicek, M., Platt, F. M., Butters, T. D., Dwek, R. A., Zimran, A.

Volltext

Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling von Neville, David C.A, Coquard, Virginie, Priestman, David A, te Vruchte, Danielle J.M, Sillence, Daniel J, Dwek, Raymond A, Platt, Frances M, Butters, Terry D

Volltext

Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C von Lachmann, Robin H, te Vruchte, Danielle, Lloyd-Evans, Emyr, Reinkensmeier, Gabriele, Sillence, Daniel J, Fernandez-Guillen, Luisa, Dwek, Raymond A, Butters, Terry D, Cox, Timothy M, Platt, Frances M

Volltext

Imino Sugars Inhibit the Formation and Secretion of Bovine Viral Diarrhea Virus, a Pestivirus Model of Hepatitis C Virus: Implications for the Development of Broad Spectrum Anti-He... von Zitzmann, Nicole, Mehta, Anand S., Carrouee, Sandra, Butters, Terry D., Platt, Frances M., McCauley, John, Blumberg, Baruch S., Dwek, Raymond A., Block, Timothy M.

Volltext

NSAIDs increase survival in the Sandhoff disease mouse: Synergy with N-butyldeoxynojirimycin von Jeyakumar, Mylvaganam, Smith, David A., Williams, Ian M., Borja, Mario Cortina, Neville, David C. A., Butters, Terry D., Dwek, Raymond A., Platt, Frances M.

Volltext

Introduction:  GlycobiologyUnderstanding the Language and Meaning of Carbohydrates von Dwek, Raymond A, Butters, Terry D

Volltext

Study of the Mechanism of Antiviral Action of Iminosugar Derivatives against Bovine Viral Diarrhea Virus von Durantel, D, Branza-Nichita, N, Carrouée-Durantel, S, Butters, T D, Dwek, R A, Zitzmann, N

Volltext

Heart failure in a woman with SLE, anti-phospholipid syndrome and Fabry’s disease von Nandagudi, A, Jury, EC, Alonzi, D, Butters, TD, Hughes, S, Isenberg, DA

Volltext

Delayed Symptom Onset and Increased Life Expectancy in Sandhoff Disease Mice Treated with N-Butyldeoxynojirimycin von Jeyakumar, Mylvaganam, Butters, Terry D., Cortina-Borja, Mario, Hunnam, Victoria, Proia, Richard L., Perry, V. Hugh, Dwek, Raymond A., Platt, Frances M.

Volltext

Reversible Infertility in Male Mice after Oral Administration of Alkylated Imino Sugars: A Nonhormonal Approach to Male Contraception von van der Spoel, Aarnoud C., Jeyakumar, Mylvaganam, Butters, Terry D., Charlton, Harry M., Moore, Harry D., Dwek, Raymond A., Platt, Frances M.

Volltext

Glucosylceramide modulates membrane traffic along the endocytic pathway von Sillence, Dan J, Puri, Vishwajeet, Marks, David L, Butters, Terry D, Dwek, Raymond A, Pagano, Richard E, Platt, Frances M

Volltext

Prevention of Lysosomal Storage in Tay-Sachs Mice Treated with N-Butyldeoxynojirimycin von Platt, Frances M., Neises, Gabrielle R., Reinkensmeier, Gabriele, Townsend, Mandy J., Perry, V. Hugh, Proia, Richard L., Winchester, Bryan, Dwek, Raymond A., Butters, Terry D.

Volltext

N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo von Andersson, Ulrika, Butters, Terry D, Dwek, Raymond A, Platt, Frances M

Volltext

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease von Andersson, Ulrika, Smith, David, Jeyakumar, Mylvaganam, Butters, Terry D, Borja, Mario Cortina, Dwek, Raymond A, Platt, Frances M

Volltext

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 S... von SUGANUMA, Ryota, WALDEN, Charlotte M, BUTTERS, Terry D, PLATT, Frances M, DWEK, Raymond A, YANAGIMACHI, Ryuzo, VAN DER SPOEL, Aarnoud C

Volltext