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Frances Platt

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Frances M Platt; Barry Boland; Aarnoud C van der Spoel
The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction.
Stefan Porubsky; Mariolina Salio; Yogesh Singh; Anneliese O Speak; Rashad Zafarulla; Mahnaz Bonrouhi; Vincenzo Cerundolo; Julian Dyson; Richard Jennemann; Agnes Lehuen; Bruno Luckow; Ernst Malle; Johannes Müthing; Frances M Platt; Hermann-Josef Gröne
Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells.
Anneliese O Speak; Daniëlle te Vruchte; Natacha Veerapen; James E Wraith; Nicole M Yanjanin; Nick Platt; Forbes D Porter; Heiko Runz; Mariolina Salio; Dawn Shepherd; Louise Simmons; David A Smith; Michael Beck; Gurdyal S Besra; Vincenzo Cerundolo; Ralf Hartung; Christian J Hendriksz; Jackie Imrie; Robin H Lachmann; Eugen Mengel; Frances M Platt
Invariant natural killer T cells are not affected by lysosomal storage in patients with Niemann-Pick disease type C.

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All Publications

2012: Frances M Platt; Barry Boland; Aarnoud C van der Spoel
The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction.
The Journal of cell biology 2012;199(5):723-34.
2012: Stefan Porubsky; Mariolina Salio; Yogesh Singh; Anneliese O Speak; Rashad Zafarulla; Mahnaz Bonrouhi; Vincenzo Cerundolo; Julian Dyson; Richard Jennemann; Agnes Lehuen; Bruno Luckow; Ernst Malle; Johannes Müthing; Frances M Platt; Hermann-Josef Gröne
Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells.
Journal of immunology (Baltimore, Md. : 1950) 2012;189(6):3007-17.
2012: Anneliese O Speak; Daniëlle te Vruchte; Natacha Veerapen; James E Wraith; Nicole M Yanjanin; Nick Platt; Forbes D Porter; Heiko Runz; Mariolina Salio; Dawn Shepherd; Louise Simmons; David A Smith; Michael Beck; Gurdyal S Besra; Vincenzo Cerundolo; Ralf Hartung; Christian J Hendriksz; Jackie Imrie; Robin H Lachmann; Eugen Mengel; Frances M Platt
Invariant natural killer T cells are not affected by lysosomal storage in patients with Niemann-Pick disease type C.
European journal of immunology 2012;42(7):1886-92.
2011: Anthony J Morgan; Frances M Platt; Emyr Lloyd-Evans; Antony Galione
Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease.
The Biochemical journal 2011;439(3):349-74.
2011: Emyr Lloyd-Evans; Frances M Platt
Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases.
Cell calcium 2011;50(2):200-5.
2011: Xuntian Jiang; Dennis J Dietzen; Hideji Fujiwara; Frances M Platt; Forbes D Porter; Jean E Schaffer; David E Scherrer; Rohini Sidhu; Anneliese O Speak; Danielle Taylor te Vruchte; Nicole M Yanjanin; Jessie Zhang; Daniel S Ory
A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma.
Journal of lipid research 2011;52(7):1435-45.
2011: Bo Pei; Anneliese O Speak; Dawn Shepherd; Terry D Butters; Vincenzo Cerundolo; Frances M Platt; Mitchell Kronenberg
Diverse endogenous antigens for mouse NKT cells: self-antigens that are not glycosphingolipids.
Journal of immunology (Baltimore, Md. : 1950) 2011;186(3):1348-60.
2010: Emyr Lloyd-Evans; Helen Waller-Evans; Ksenia Peterneva; Frances M Platt
Endolysosomal calcium regulation and disease.
Biochemical Society transactions 2010;38(6):1458-64.
2010: Einat B Vitner; Frances M Platt; Anthony H Futerman
Common and uncommon pathogenic cascades in lysosomal storage diseases.
The Journal of biological chemistry 2010;285(27):20423-7.
2010: Daniëlle te Vruchte; Aruna Jeans; Frances M Platt; Dan J Sillence
Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice.
Journal of inherited metabolic disease 2010;33(3):261-70.
2010: Christine A Denny; Karie A Heinecke; Youngho P Kim; Rena C Baek; Katrina S Loh; Terry D Butters; Roderick T Bronson; Frances M Platt; Thomas N Seyfried
Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice.
Journal of neurochemistry 2010;113(6):1525-35.
2010: Margarida Ruas; John A Ward; Keiko Watanabe; Gary M Wessel; Michael X Zhu; Abdelilah Arredouani; Xiaotong Cheng; Grant C Churchill; Lianne C Davis; Timothy M Funnell; Heidi Koegel; Emyr Lloyd-Evans; Anthony J Morgan; John Parrington; Frances M Platt; Katja Rietdorf; Antony Galione
Purified TPC isoforms form NAADP receptors with distinct roles for Ca(2+) signaling and endolysosomal trafficking.
Current biology : CB 2010;20(8):703-9.
2010: Emyr Lloyd-Evans; Frances M Platt
Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease.
Traffic (Copenhagen, Denmark) 2010;11(4):419-28.
2009: David Smith; Kerri-Lee Wallom; Ian M Williams; Mylvaganam Jeyakumar; Frances M Platt
Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1.
Neurobiology of disease 2009;36(2):242-51.
2009: Mylvaganam Jeyakumar; Jean-Pyo Lee; Nicola R Sibson; John P Lowe; Daniel J Stuckey; Katie Tester; Gerald Fu; Robin Newlin; David A Smith; Evan Y Snyder; Frances M Platt
Neural stem cell transplantation benefits a monogenic neurometabolic disorder during the symptomatic phase of disease.
Stem cells (Dayton, Ohio) 2009;27(9):2362-70.
2009: Mylvaganam Jeyakumar; Ian Williams; David Smith; Timothy M Cox; Frances M Platt
Critical role of iron in the pathogenesis of the murine gangliosidoses.
Neurobiology of disease 2009;34(3):406-16.
2009: Frances M Platt; Robin H Lachmann
Treating lysosomal storage disorders: current practice and future prospects.
Biochimica et biophysica acta 2009;1793(4):737-45.
2009: Frances M Platt; Robin H Lachmann
A new surrogate marker for CNS pathology in Niemann-Pick disease type C?
Molecular genetics and metabolism 2009;96(2):53-4.
2008: Carmela De Santo; Mariolina Salio; S Hajar Masri; Laurel Yong-Hwa Lee; Tao Dong; Anneliese O Speak; Stefan Porubsky; Sarah Booth; Natacha Veerapen; Gurdyal S Besra; Hermann-Josef Gröne; Frances M Platt; Maria Zambon; Vincenzo Cerundolo
Invariant NKT cells reduce the immunosuppressive activity of influenza A virus-induced myeloid-derived suppressor cells in mice and humans.
The Journal of clinical investigation 2008;118(12):4036-48.
2008: Emyr Lloyd-Evans; Anthony J Morgan; Xingxuan He; David A Smith; Elena Elliot-Smith; Dan J Sillence; Grant C Churchill; Edward H Schuchman; Antony Galione; Frances M Platt
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium.
Nature medicine 2008;14(11):1247-55.
2008: Stefan Porubsky; Bruno Luckow; Mahnaz Bonrouhi; A Speak; Vincenzo Cerundolo; Frances M Platt; Hermann-Josef Gröne
[Glycosphingolipids Gb3 and iGb3. In vivo roles in hemolytic-uremic syndrome and iNKT cell function].
Der Pathologe 2008;29 Suppl 2():297-302.
2008: Kate H Townson; Anneliese O Speak; Kay N Greenshields; Carl S Goodyear; Hugh J Willison; Frances M Platt
Glycosphingolipid depletion in PC12 cells using iminosugars protects neuronal membranes from anti-ganglioside antibody mediated injury.
Journal of neuroimmunology 2008;203(1):33-8.
2008: Anneliese O Speak; Vincenzo Cerundolo; Frances M Platt
CD1d presentation of glycolipids.
Immunology and cell biology 2008;86(7):588-97.
2008: Aarnoud C van der Spoel; Richard Mott; Frances M Platt
Differential sensitivity of mouse strains to an N-alkylated imino sugar: glycosphingolipid metabolism and acrosome formation.
Pharmacogenomics 2008;9(6):717-31.
2008: Elena Elliot-Smith; Anneliese O Speak; Emyr Lloyd-Evans; David A Smith; Aarnoud C van der Spoel; Mylvaganam Jeyakumar; Terry D Butters; Raymond A Dwek; Alessandra d'Azzo; Frances M Platt
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis.
Molecular genetics and metabolism 2008;94(2):204-11.
2008: Mariona Rabionet; Aarnoud C van der Spoel; Chia-Chen Chuang; Benita von Tümpling-Radosta; Manja Litjens; Diane Bouwmeester; Christina C Hellbusch; Christian Körner; Herbert Wiegandt; Karin Gorgas; Frances M Platt; Hermann-Josef Gröne; Roger Sandhoff
Male germ cells require polyenoic sphingolipids with complex glycosylation for completion of meiosis: a link to ceramide synthase-3.
The Journal of biological chemistry 2008;283(19):13357-69.
2008: Rena C Baek; Julie L Kasperzyk; Frances M Platt; Thomas N Seyfried
N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice.
Neurochemistry international 2008;52(6):1125-33.
2008: Frances M Platt; Mylvaganam Jeyakumar
Substrate reduction therapy.
Acta paediatrica (Oslo, Norway : 1992). Supplement 2008;97(457):88-93.
2008: David A Priestman; Aarnoud C van der Spoel; Terry D Butters; Raymond A Dwek; Frances M Platt
N-butyldeoxynojirimycin causes weight loss as a result of appetite suppression in lean and obese mice.
Diabetes, obesity & metabolism 2008;10(2):159-66.
2007: Mariolina Salio; Anneliese O Speak; Dawn Shepherd; Paolo Polzella; Petr A Illarionov; Natacha Veerapen; Gurdyal S Besra; Frances M Platt; Vincenzo Cerundolo
Modulation of human natural killer T cell ligands on TLR-mediated antigen-presenting cell activation.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(51):20490-5.
2007: Alexander T H Wu; Peter Sutovsky; Wei Xu; Aarnoud C van der Spoel; Frances M Platt; Richard Oko
The postacrosomal assembly of sperm head protein, PAWP, is independent of acrosome formation and dependent on microtubular manchette transport.
Developmental biology 2007;312(2):471-83.
2007: Charlotte M Walden; Roger Sandhoff; Chia-Chen Chuang; Yildiz Yildiz; Terry D Butters; Raymond A Dwek; Frances M Platt; Aarnoud C van der Spoel
Accumulation of glucosylceramide in murine testis, caused by inhibition of beta-glucosidase 2: implications for spermatogenesis.
The Journal of biological chemistry 2007;282(45):32655-64.
2007: Christophe Paget; Thierry Mallevaey; Anneliese O Speak; David Torres; Josette Fontaine; Kathleen C F Sheehan; Monique Capron; Bernhard RYFFEL; Christelle Faveeuw; Maria C Leite-de-Moraes; Frances M Platt; François Trottein
Activation of invariant NKT cells by toll-like receptor 9-stimulated dendritic cells requires type I interferon and charged glycosphingolipids.
Immunity 2007;27(4):597-609.
2007: Giovanni D'Angelo; Elena V Polishchuk; Giuseppe Di Tullio; Michele Santoro; Antonella Di Campli; Anna Godi; Gun West; Jacek Bielawski; Chia-Chen Chuang; Aarnoud C van der Spoel; Frances M Platt; Yusuf A Hannun; Roman S Polishchuk; Peter Mattjus; Maria Antonietta De Matteis
Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide.
Nature 2007;449(7158):62-7.
2007: Anneliese O Speak; Mariolina Salio; David C A Neville; Josette Fontaine; David A Priestman; Nick Platt; Tanya Heare; Terry D Butters; Raymond A Dwek; François Trottein; Mark A Exley; Vincenzo Cerundolo; Frances M Platt
Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(14):5971-6.
2007: Stefan Porubsky; Anneliese O Speak; Bruno Luckow; Vincenzo Cerundolo; Frances M Platt; Hermann-Josef Gröne
Normal development and function of invariant natural killer T cells in mice with isoglobotrihexosylceramide (iGb3) deficiency.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(14):5977-82.
2007: Jean-Pyo Lee; Mylvaganam Jeyakumar; Rodolfo Gonzalez; Hiroto Takahashi; Pei-Jen Lee; Rena C Baek; Dan Clark; Heather Rose; Gerald Fu; Jonathan Clarke; Scott R McKercher; Jennifer Meerloo; Franz-Josef Muller; Kook In Park; Terry D Butters; Raymond A Dwek; Philip Schwartz; Gang Tong; David Wenger; Stuart A Lipton; Thomas N Seyfried; Frances M Platt; Evan Y Snyder
Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease.
Nature medicine 2007;13(4):439-47.
2007: Tanya Heare; Nicholas J Alp; David A Priestman; Ashok Kulkarni; Pankaj Qasba; Terry D Butters; Raymond A Dwek; K Clarke; K M Channon; Frances M Platt
Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment.
Journal of inherited metabolic disease 2007;30(1):79-87.
2007: Wilhelm Bone; Charlotte M Walden; Martin Fritsch; Ulrike Voigtmann; Eckhard Leifke; Ulrich Gottwald; Stephanie Boomkamp; Frances M Platt; Aarnoud C van der Spoel
The sensitivity of murine spermiogenesis to miglustat is a quantitative trait: a pharmacogenetic study.
Reproductive biology and endocrinology : RB&E 2007;5():1.
2006: Stephan D Gadola; Jonathan Silk; Aruna Jeans; Petr A Illarionov; Mariolina Salio; Gurdyal S Besra; Raymond A Dwek; Terry D Butters; Frances M Platt; Vincenzo Cerundolo
Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases.
The Journal of experimental medicine 2006;203(10):2293-303.
2006: Charlotte M Walden; Terry D Butters; Raymond A Dwek; Frances M Platt; Aarnoud C van der Spoel
Long-term non-hormonal male contraception in mice using N-butyldeoxynojirimycin.
Human reproduction (Oxford, England) 2006;21(5):1309-15.
2006: Daniel C Smith; Dan J Sillence; Thomas Falguières; Rosemary M Jarvis; Ludger Johannes; J Michael Lord; Frances M Platt; Lynne M Roberts
The association of Shiga-like toxin with detergent-resistant membranes is modulated by glucosylceramide and is an essential requirement in the endoplasmic reticulum for a cytotoxic effect.
Molecular biology of the cell 2006;17(3):1375-87.
2006: Thierry Mallevaey; Jean-Pierre Zanetta; Christelle Faveeuw; Josette Fontaine; Emmanuel Maes; Frances M Platt; Monique Capron; Maria Leite- de-Moraes; François Trottein
Activation of invariant NKT cells by the helminth parasite schistosoma mansoni.
Journal of immunology (Baltimore, Md. : 1950) 2006;176(4):2476-85.
2005: John P Lowe; Daniel J Stuckey; F R Awan; Mylvaganam Jeyakumar; David C A Neville; Frances M Platt; Julian L Griffin; Peter Styles; Andrew M Blamire; Nicola R Sibson
MRS reveals additional hexose N-acetyl resonances in the brain of a mouse model for Sandhoff disease.
NMR in biomedicine 2005;18(8):517-26.
2005: Terry D Butters; Raymond A Dwek; Frances M Platt
Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.
Glycobiology 2005;15(10):43R-52R.
2005: Mylvaganam Jeyakumar; Raymond A Dwek; Terry D Butters; Frances M Platt
Storage solutions: treating lysosomal disorders of the brain.
Nature reviews. Neuroscience 2005;6(9):713-25.
2005: Ryota Suganuma; Charlotte M Walden; Terry D Butters; Frances M Platt; Raymond A Dwek; Ryuzo Yanagimachi; Aarnoud C van der Spoel
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 2005;72(4):805-13.
2005: Frances M Platt; Mylvaganam Jeyakumar; Ulrika Andersson; Raymond A Dwek; Terry D Butters
New developments in treating glycosphingolipid storage diseases.
Advances in experimental medicine and biology 2005;564():117-26.
2004: Mylvaganam Jeyakumar; A David Smith; Ian M Williams; Mario Cortina Borja; David C A Neville; Terry D Butters; Raymond A Dwek; Frances M Platt
NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin.
Annals of neurology 2004;56(5):642-9.
2004: Michael A Simpson; Harold Cross; christos proukakis; David A Priestman; David C A Neville; Gabriele Reinkensmeier; Heng Wang; Max Wiznitzer; Kay Gurtz; Argyro Verganelaki; Anna Pryde; Michael A Patton; Raymond A Dwek; Terry D Butters; Frances M Platt; Andrew H Crosby
Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase.
Nature genetics 2004;36(11):1225-9.
2004: Edward Norris-Cervetto; Richard Callaghan; Frances M Platt; Raymond A Dwek; Terry D Butters
Inhibition of glucosylceramide synthase does not reverse drug resistance in cancer cells.
The Journal of biological chemistry 2004;279(39):40412-8.
2004: Gang Tian; David Wilcockson; V Hugh Perry; Pauline M Rudd; Raymond A Dwek; Frances M Platt; Nick Platt
Inhibition of alpha-glucosidases I and II increases the cell surface expression of functional class A macrophage scavenger receptor (SR-A) by extending its half-life.
The Journal of biological chemistry 2004;279(38):39303-9.
2004: David C A Neville; Virginie Coquard; David A Priestman; Daniëlle te Vruchte; Dan J Sillence; Raymond A Dwek; Frances M Platt; Terry D Butters
Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling.
Analytical biochemistry 2004;331(2):275-82.
2004: Robin H Lachmann; Daniëlle te Vruchte; Emyr Lloyd-Evans; Gabriele Reinkensmeier; Dan J Sillence; Luisa Fernandez-Guillen; Raymond A Dwek; Terry D Butters; Timothy M Cox; Frances M Platt
Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C.
Neurobiology of disease 2004;16(3):654-8.
2004: Ulrika Andersson; David Smith; Mylvaganam Jeyakumar; Terry D Butters; Mario Cortina Borja; Raymond A Dwek; Frances M Platt
Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease.
Neurobiology of disease 2004;16(3):506-15.
2004: Dan J Sillence; Frances M Platt
Glycosphingolipids in endocytic membrane transport.
Seminars in cell & developmental biology 2004;15(4):409-16.
2004: Dan J Sillence; Frances M Platt
Introduction: Glycosphingolipids in cell biology and disease.
Seminars in cell & developmental biology 2004;15(4):371-3.
2004: Howard R Mellor; David C A Neville; David Harvey; Frances M Platt; Raymond A Dwek; Terry D Butters
Cellular effects of deoxynojirimycin analogues: inhibition of N-linked oligosaccharide processing and generation of free glucosylated oligosaccharides.
The Biochemical journal 2004;381(Pt 3):867-75.
2004: Howard R Mellor; David C A Neville; David Harvey; Frances M Platt; Raymond A Dwek; Terry D Butters
Cellular effects of deoxynojirimycin analogues: uptake, retention and inhibition of glycosphingolipid biosynthesis.
The Biochemical journal 2004;381(Pt 3):861-6.
2004: Daniëlle te Vruchte; Emyr Lloyd-Evans; Robert Jan Veldman; David C A Neville; Raymond A Dwek; Frances M Platt; Wim J van Blitterswijk; Dan J Sillence
Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport.
The Journal of biological chemistry 2004;279(25):26167-75.
2004: Ulrika Andersson; Gabriele Reinkensmeier; Terry D Butters; Raymond A Dwek; Frances M Platt
Inhibition of glycogen breakdown by imino sugars in vitro and in vivo.
Biochemical pharmacology 2004;67(4):697-705.
2004: Deborah Elstein; Carla E M Hollak; johannes aerts; Sonja van Weely; M Maas; Timothy M Cox; Robin H Lachmann; Martin Hrebicek; Frances M Platt; Terry D Butters; Raymond A Dwek; Ari Zimran
Sustained therapeutic effects of oral miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) in type I Gaucher disease.
Journal of inherited metabolic disease 2004;27(6):757-66.
2003: Howard R Mellor; Frances M Platt; Raymond A Dwek; Terry D Butters
Membrane disruption and cytotoxicity of hydrophobic N-alkylated imino sugars is independent of the inhibition of protein and lipid glycosylation.
The Biochemical journal 2003;374(Pt 2):307-14.
2003: Dori Pelled; Emyr Lloyd-Evans; Christian Riebeling; Mylvaganam Jeyakumar; Frances M Platt; Anthony H Futerman
Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.
The Journal of biological chemistry 2003;278(32):29496-501.
2003: Frances M Platt; Mylvaganam Jeyakumar; Ulrika Andersson; Tanya Heare; Raymond A Dwek; Terry D Butters
Substrate reduction therapy in mouse models of the glycosphingolipidoses.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences 2003;358(1433):947-54.
2003: Terry D Butters; Howard R Mellor; Keishi Narita; Raymond A Dwek; Frances M Platt
Small-molecule therapeutics for the treatment of glycolipid lysosomal storage disorders.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences 2003;358(1433):927-45.
2003: Dan J Sillence; Frances M Platt
Storage diseases: new insights into sphingolipid functions.
Trends in cell biology 2003;13(4):195-203.
2003: Mylvaganam Jeyakumar; R Thomas; E Elliot-Smith; D A Smith; Aarnoud C van der Spoel; Alessandra d'Azzo; V Hugh Perry; Terry D Butters; Raymond A Dwek; Frances M Platt
Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis.
Brain : a journal of neurology 2003;126(Pt 4):974-87.
2003: Terry D Butters; Raymond A Dwek; Frances M Platt
New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases.
Advances in experimental medicine and biology 2003;535():219-26.
2003: Terry D Butters; Raymond A Dwek; Frances M Platt
Therapeutic applications of imino sugars in lysosomal storage disorders.
Current topics in medicinal chemistry 2003;3(5):561-74.
2002: Aarnoud C van der Spoel; Mylvaganam Jeyakumar; Terry D Butters; Harry M Charlton; Harry D Moore; Raymond A Dwek; Frances M Platt
Reversible infertility in male mice after oral administration of alkylated imino sugars: a nonhormonal approach to male contraception.
Proceedings of the National Academy of Sciences of the United States of America 2002;99(26):17173-8.
2002: Dan J Sillence; Vishwajeet Puri; David L Marks; Terry D Butters; Raymond A Dwek; Richard E Pagano; Frances M Platt
Glucosylceramide modulates membrane traffic along the endocytic pathway.
Journal of lipid research 2002;43(11):1837-45.
2002: Mylvaganam Jeyakumar; Terry D Butters; Raymond A Dwek; Frances M Platt
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
Neuropathology and applied neurobiology 2002;28(5):343-57.
2002: Howard R Mellor; James Nolan; Lea Pickering; Mark R Wormald; Frances M Platt; Raymond A Dwek; George W J Fleet; Terry D Butters
Preparation, biochemical characterization and biological properties of radiolabelled N-alkylated deoxynojirimycins.
The Biochemical journal 2002;366(Pt 1):225-33.
2002: Mylvaganam Jeyakumar; David Smith; Elena Eliott-Smith; Mario Cortina Borja; Gabriele Reinkensmeier; Terry D Butters; Thorsten Lemm; Konrad Sandhoff; V Hugh Perry; Raymond A Dwek; Frances M Platt
An inducible mouse model of late onset Tay-Sachs disease.
Neurobiology of disease 2002;10(3):201-10.
2002: Brett Garner; Howard R Mellor; Terry D Butters; Raymond A Dwek; Frances M Platt
Modulation of THP-1 macrophage and cholesterol-loaded foam cell apolipoprotein E levels by glycosphingolipids.
Biochemical and biophysical research communications 2002;290(5):1361-7.
2002: Brett Garner; David A Priestman; Roland Stocker; David Harvey; Terry D Butters; Frances M Platt
Increased glycosphingolipid levels in serum and aortae of apolipoprotein E gene knockout mice.
Journal of lipid research 2002;43(2):205-14.
2002: Raymond A Dwek; Terry D Butters; Frances M Platt; Nicole Zitzmann
Targeting glycosylation as a therapeutic approach.
Nature reviews. Drug discovery 2002;1(1):65-75.
2001: D R Wing; Brett Garner; V Hunnam; Gabriele Reinkensmeier; Ulrika Andersson; David Harvey; Raymond A Dwek; Frances M Platt; Terry D Butters
High-performance liquid chromatography analysis of ganglioside carbohydrates at the picomole level after ceramide glycanase digestion and fluorescent labeling with 2-aminobenzamide.
Analytical biochemistry 2001;298(2):207-17.
2001: Frances M Platt; Mylvaganam Jeyakumar; Ulrika Andersson; David A Priestman; Raymond A Dwek; Terry D Butters; Timothy M Cox; Robin H Lachmann; Carla E M Hollak; johannes aerts; Sonja van Weely; M Hrebícek; C Moyses; I Gow; Deborah Elstein; Ari Zimran
Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy.
Journal of inherited metabolic disease 2001;24(2):275-90.
2001: Robin H Lachmann; Frances M Platt
Substrate reduction therapy for glycosphingolipid storage disorders.
Expert opinion on investigational drugs 2001;10(3):455-66.
2001: Mylvaganam Jeyakumar; F Norflus; Cynthia J Tifft; Mario Cortina Borja; Terry D Butters; Richard L Proia; V Hugh Perry; Raymond A Dwek; Frances M Platt
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.
Blood 2001;97(1):327-9.
2000: David A Priestman; Frances M Platt; Raymond A Dwek; Terry D Butters
Imino sugar therapy for type 1 Gaucher disease.
Glycobiology 2000;10(11):iv-vi.
2000: Howard R Mellor; A Adam; Frances M Platt; Raymond A Dwek; Terry D Butters
High-performance cation-exchange chromatography and pulsed amperometric detection for the separation, detection, and quantitation of N-alkylated imino sugars in biological samples.
Analytical biochemistry 2000;284(1):136-42.
2000: T Cox; Robin H Lachmann; Carla E M Hollak; johannes aerts; Sonja van Weely; M Hrebícek; Frances M Platt; Terry D Butters; Raymond A Dwek; C Moyses; I Gow; Deborah Elstein; Ari Zimran
Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.
Lancet 2000;355(9214):1481-5.
2000: Ulrika Andersson; Terry D Butters; Raymond A Dwek; Frances M Platt
N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo.
Biochemical pharmacology 2000;59(7):821-9.
2000: Norica Branza-Nichita; Gabriela Negroiu; Andrei J Petrescu; E F Garman; Frances M Platt; Mark R Wormald; Raymond A Dwek; Stefana M Petrescu
Mutations at critical N-glycosylation sites reduce tyrosinase activity by altering folding and quality control.
The Journal of biological chemistry 2000;275(11):8169-75.
1999: Nicole Zitzmann; Anand S Mehta; S Carrouée; Terry D Butters; Frances M Platt; J McCauley; Baruch S Blumberg; Raymond A Dwek; Timothy M Block
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-hepatitis virus agents.
Proceedings of the National Academy of Sciences of the United States of America 1999;96(21):11878-82.
1999: Norica Branza-Nichita; Andrei J Petrescu; Raymond A Dwek; Mark R Wormald; Frances M Platt; Stefana M Petrescu
Tyrosinase folding and copper loading in vivo: a crucial role for calnexin and alpha-glucosidase II.
Biochemical and biophysical research communications 1999;261(3):720-5.
1999: Mylvaganam Jeyakumar; Terry D Butters; Mario Cortina Borja; V Hunnam; Richard L Proia; V Hugh Perry; Raymond A Dwek; Frances M Platt
Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.
Proceedings of the National Academy of Sciences of the United States of America 1999;96(11):6388-93.
1998: Frances M Platt; Terry D Butters
New therapeutic prospects for the glycosphingolipid lysosomal storage diseases.
Biochemical pharmacology 1998;56(4):421-30.
1997: Frances M Platt; Gabriele Reinkensmeier; Raymond A Dwek; Terry D Butters
Extensive glycosphingolipid depletion in the liver and lymphoid organs of mice treated with N-butyldeoxynojirimycin.
The Journal of biological chemistry 1997;272(31):19365-72.
1997: Andrei J Petrescu; Terry D Butters; Gabriele Reinkensmeier; Stefana M Petrescu; Frances M Platt; Raymond A Dwek; Mark R Wormald
The solution NMR structure of glucosylated N-glycans involved in the early stages of glycoprotein biosynthesis and folding.
The EMBO journal 1997;16(14):4302-10.
1997: Stefana M Petrescu; Andrei J Petrescu; H N Titu; Raymond A Dwek; Frances M Platt
Inhibition of N-glycan processing in B16 melanoma cells results in inactivation of tyrosinase but does not prevent its transport to the melanosome.
The Journal of biological chemistry 1997;272(25):15796-803.
1997: Frances M Platt; G R Neises; Gabriele Reinkensmeier; MJ Townsend; V Hugh Perry; Richard L Proia; B Winchester; Raymond A Dwek; Terry D Butters
Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin.
Science (New York, N.Y.) 1997;276(5311):428-31.
1996: P B Fischer; G B Karlsson; Raymond A Dwek; Frances M Platt
N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with impaired gp120 shedding and gp41 exposure.
Journal of virology 1996;70(10):7153-60.
1996: P B Fischer; G B Karlsson; Terry D Butters; Raymond A Dwek; Frances M Platt
N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120.
Journal of virology 1996;70(10):7143-52.
1995: P B Fischer; M Collin; G B Karlsson; William James; Terry D Butters; Simon J Davis; Siamon Gordon; Raymond A Dwek; Frances M Platt
The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding.
Journal of virology 1995;69(9):5791-7.
1994: Frances M Platt; G R Neises; G B Karlsson; Raymond A Dwek; Terry D Butters
N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing.
The Journal of biological chemistry 1994;269(43):27108-14.
1994: Frances M Platt; G R Neises; Raymond A Dwek; Terry D Butters
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.
The Journal of biological chemistry 1994;269(11):8362-5.
1994: Timothy M Block; X Lu; Frances M Platt; G R Foster; W H Gerlich; Baruch S Blumberg; Raymond A Dwek
Secretion of human hepatitis B virus is inhibited by the imino sugar N-butyldeoxynojirimycin.
Proceedings of the National Academy of Sciences of the United States of America 1994;91(6):2235-9.
1993: Iain Wilson; Frances M Platt; D A Isenberg; T W Rademacher
Aberrant control of galactosyltransferase in peripheral B lymphocytes and Epstein-Barr virus transformed B lymphoblasts from patients with rheumatoid arthritis.
The Journal of rheumatology 1993;20(8):1282-7.
1993: G B Karlsson; Terry D Butters; Raymond A Dwek; Frances M Platt
Effects of the imino sugar N-butyldeoxynojirimycin on the N-glycosylation of recombinant gp120.
The Journal of biological chemistry 1993;268(1):570-6.
1992: Frances M Platt; G B Karlsson; Gary S Jacob
Modulation of cell-surface transferrin receptor by the imino sugar N-butyldeoxynojirimycin.
European journal of biochemistry / FEBS 1992;208(1):187-93.
1991: G B Karlsson; Frances M Platt
Analysis and isolation of human transferrin receptor using the OKT-9 monoclonal antibody covalently crosslinked to magnetic beads.
Analytical biochemistry 1991;199(2):219-22.