Terry D Butters

Summary

Affiliation: University of Oxford
Country: UK

Publications

  1. doi request reprint Novel mannosidase inhibitors probe glycoprotein degradation pathways in cells
    Terry D Butters
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Glycoconj J 26:1109-16. 2009
  2. ncbi request reprint Gaucher disease
    Terry D Butters
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Curr Opin Chem Biol 11:412-8. 2007
  3. ncbi request reprint Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses
    Terry D Butters
    Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Glycobiology 15:43R-52R. 2005
  4. ncbi request reprint Pharmacotherapeutic strategies using small molecules for the treatment of glycolipid lysosomal storage disorders
    Terry D Butters
    Oxford University, Oxford Glycobiology Institute, Department of Biochemistry, South Parks Road, Oxford, OX1 3QU, UK
    Expert Opin Pharmacother 8:427-35. 2007
  5. doi request reprint The conformational properties of the Glc3Man unit suggest conformational biasing within the chaperone-assisted glycoprotein folding pathway
    Mukram M Mackeen
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    J Mol Biol 387:335-47. 2009
  6. pmc Cellular effects of deoxynojirimycin analogues: inhibition of N-linked oligosaccharide processing and generation of free glucosylated oligosaccharides
    Howard R Mellor
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 381:867-75. 2004
  7. doi request reprint Glycoprotein misfolding in the endoplasmic reticulum: identification of released oligosaccharides reveals a second ER-associated degradation pathway for Golgi-retrieved proteins
    Dominic S Alonzi
    Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Cell Mol Life Sci 70:2799-814. 2013
  8. doi request reprint Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis
    Elena Elliot-Smith
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Mol Genet Metab 94:204-11. 2008
  9. ncbi request reprint Glucosylated free oligosaccharides are biomarkers of endoplasmic- reticulum alpha-glucosidase inhibition
    Dominic S Alonzi
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 409:571-80. 2008
  10. pmc Membrane disruption and cytotoxicity of hydrophobic N-alkylated imino sugars is independent of the inhibition of protein and lipid glycosylation
    Howard R Mellor
    Department of Biochemistry, Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 374:307-14. 2003

Collaborators

Detail Information

Publications65

  1. doi request reprint Novel mannosidase inhibitors probe glycoprotein degradation pathways in cells
    Terry D Butters
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Glycoconj J 26:1109-16. 2009
    ..The availability of more selective inhibitors allows the pathways of N-linked oligosaccharide metabolism to be dissected...
  2. ncbi request reprint Gaucher disease
    Terry D Butters
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Curr Opin Chem Biol 11:412-8. 2007
    ..This novel, chaperon-mediated approach has benefited from insights into the molecular understanding of beta-glucocerebrosidase structure, drug design and development in cellular models for disease...
  3. ncbi request reprint Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses
    Terry D Butters
    Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Glycobiology 15:43R-52R. 2005
    ..This is particularly so given the ability of small molecules to be orally available, penetrate the central nervous system (CNS), and have well-characterized biological and pharmacological properties...
  4. ncbi request reprint Pharmacotherapeutic strategies using small molecules for the treatment of glycolipid lysosomal storage disorders
    Terry D Butters
    Oxford University, Oxford Glycobiology Institute, Department of Biochemistry, South Parks Road, Oxford, OX1 3QU, UK
    Expert Opin Pharmacother 8:427-35. 2007
    ..The advantages of using small molecules as therapy for the family of lysosomal storage disorders are discussed with reference to existing enzyme replacement therapies...
  5. doi request reprint The conformational properties of the Glc3Man unit suggest conformational biasing within the chaperone-assisted glycoprotein folding pathway
    Mukram M Mackeen
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    J Mol Biol 387:335-47. 2009
    ..This is the first time evidence has been presented on glycoprotein folding that suggests the process may be optimized to balance the chaperone-assisted and chaperone-independent pathways...
  6. pmc Cellular effects of deoxynojirimycin analogues: inhibition of N-linked oligosaccharide processing and generation of free glucosylated oligosaccharides
    Howard R Mellor
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 381:867-75. 2004
    ..This represents the most detailed characterization of the FOS structures arising from ER a-glucosidase inhibition to date...
  7. doi request reprint Glycoprotein misfolding in the endoplasmic reticulum: identification of released oligosaccharides reveals a second ER-associated degradation pathway for Golgi-retrieved proteins
    Dominic S Alonzi
    Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Cell Mol Life Sci 70:2799-814. 2013
    ..The production of such lumenal FOS is indicative of a novel degradative route for cellular glycoproteins that may exist under certain conditions. ..
  8. doi request reprint Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis
    Elena Elliot-Smith
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Mol Genet Metab 94:204-11. 2008
    ..However, functional improvement was greatest with NB-DNJ treatment which may potentially be caused by novel anti-inflammatory properties of NB-DNJ...
  9. ncbi request reprint Glucosylated free oligosaccharides are biomarkers of endoplasmic- reticulum alpha-glucosidase inhibition
    Dominic S Alonzi
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 409:571-80. 2008
    ..In mouse and human urine, glucosylated FOS were detected as a result of transrenal excretion and provide unique and quantifiable biomarkers of ER-glucosidase inhibition...
  10. pmc Membrane disruption and cytotoxicity of hydrophobic N-alkylated imino sugars is independent of the inhibition of protein and lipid glycosylation
    Howard R Mellor
    Department of Biochemistry, Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 374:307-14. 2003
    ..This information will aid in the future development of more selective imino sugar therapeutics for the treatment of human disease...
  11. ncbi request reprint An approach to 8 stereoisomers of homonojirimycin from (D)-glucose via kinetic & thermodynamic azido-γ-lactones
    Andreas F G Glawar
    Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Org Biomol Chem 11:6886-99. 2013
    ..A side-by-side glycosidase inhibition profile of 11 of the possible 16 HNJ stereoisomers derived from d-glucose and d-mannose is presented. ..
  12. doi request reprint Demonstration that endoplasmic reticulum-associated degradation of glycoproteins can occur downstream of processing by endomannosidase
    Nikolay V Kukushkin
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 438:133-42. 2011
    ..Using this approach, we have identified a previously unknown pathway of glycoprotein flow, undetectable by the commonly employed methods, in which secretory cargo is targeted back to the ER after being processed by endomannosidase...
  13. doi request reprint Lysosomal storage of oligosaccharide and glycosphingolipid in imino sugar treated cells
    Stephanie D Boomkamp
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Glycoconj J 27:297-308. 2010
    ..Using a chemically induced gangliosidosis phenotype that can be modulated with substrate lowering drugs, the critical role of GM2 ganglioside in the progression of inflammatory disease is also demonstrated...
  14. doi request reprint 3-Hydroxyazetidine carboxylic acids: non-proteinogenic amino acids for medicinal chemists
    Andreas F G Glawar
    Chemistry Research Laboratory, Department of Chemistry, University of Oxford, Mansfield Road, Oxford, OX1 3TA, UK
    ChemMedChem 8:658-66. 2013
    ....
  15. ncbi request reprint Inhibition of glucosylceramide synthase does not reverse drug resistance in cancer cells
    Edward Norris-Cervetto
    Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    J Biol Chem 279:40412-8. 2004
    ..Our results suggest that (a) inhibition of glucosylceramide synthase does not reverse multidrug resistance and (b) the chemosensitization achieved by PDMP cannot be caused by inhibition of glucosylceramide synthase alone...
  16. ncbi request reprint NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin
    Mylvaganam Jeyakumar
    Glycobiology Institute, Department of Biochemistry, University of Oxford, Oxford, OX1 3QU
    Ann Neurol 56:642-9. 2004
    ..00001). This study demonstrates that inflammation contributes to disease progression and identifies antiinflammatory and antioxidant therapies as a potential adjunctive approach to slow the clinical course of this and related disorders...
  17. doi request reprint Synthesis and biological characterisation of novel N-alkyl-deoxynojirimycin alpha-glucosidase inhibitors
    Amy J Rawlings
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, UK
    Chembiochem 10:1101-5. 2009
    ....
  18. ncbi request reprint Long-term non-hormonal male contraception in mice using N-butyldeoxynojirimycin
    Charlotte M Walden
    The Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, Oxford, UK
    Hum Reprod 21:1309-15. 2006
    ..As contraceptives need to be taken for extended periods of time, it was essential to evaluate NB-DNJ for its reproductive effects over a long period of administration...
  19. ncbi request reprint Can P-glycoprotein influence the bioavailability of iminosugar-based glucosylceramide synthase inhibitors?
    Edward Norris-Cervetto
    Nuffield Department of Clinical Laboratory Sciences, Level 4, John Radcliffe Hospital, Oxford, OX3 9DU, UK
    Eur J Pharmacol 530:195-204. 2006
    ..Consequently, P-glycoprotein expression will not contribute significantly to the pharmacokinetic profile of the iminosugar glucosylceramide synthase inhibitors...
  20. ncbi request reprint The importance of including local correlation times in the calculation of inter-proton distances from NMR measurements: ignoring local correlation times leads to significant errors in the conformational analysis of the Glc alpha1-2Glc alpha linkage by NMR
    Mukram Mackeen
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Org Biomol Chem 4:2241-6. 2006
    ..The inter-proton distances calculated including the effects of differences in local correlation times give much more consistent results...
  21. ncbi request reprint Accumulation of glucosylceramide in murine testis, caused by inhibition of beta-glucosidase 2: implications for spermatogenesis
    Charlotte M Walden
    Departments of Biochemistry and Pharmacology, University of Oxford, Mansfield Road, Oxford, United Kingdom
    J Biol Chem 282:32655-64. 2007
    ..Therefore, it appears that acrosome formation can be derailed by accumulation of glucosylceramide in an extralysosomal localization, and that the sensitivity of male germ cells to glucosylceramide is genetically determined...
  22. doi request reprint Design, synthesis, and biological evaluation of enantiomeric beta-N-acetylhexosaminidase inhibitors LABNAc and DABNAc as potential agents against Tay-Sachs and Sandhoff disease
    J S Shane Rountree
    Chemistry Research Laboratory, Department of Chemistry, University of Oxford, Mansfield Road, Oxford, OX1 3TA, UK
    ChemMedChem 4:378-92. 2009
    ..LABNAc, NBn-LABNAc, and NBu-LABNAc are potent and selective inhibitors of beta-N-acetylhexosaminidase and may be useful as therapeutic agents for treating adult Tay-Sachs and Sandhoff diseases...
  23. ncbi request reprint Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease
    Ulrika Andersson
    Department of Biochemistry, Glycobiology Institute, University of Oxford, Oxford OX1 3QU, UK
    Neurobiol Dis 16:506-15. 2004
    ..The ability to escalate the dose of NB-DGJ, leading to extended life expectancy and increased delay in symptom onset, demonstrates the greater therapeutic potential of NB-DGJ for the treatment of the human gangliosidoses...
  24. pmc Cellular effects of deoxynojirimycin analogues: uptake, retention and inhibition of glycosphingolipid biosynthesis
    Howard R Mellor
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem J 381:861-6. 2004
    ....
  25. doi request reprint C-branched iminosugars: α-glucosidase inhibition by enantiomers of isoDMDP, isoDGDP, and isoDAB-L-isoDMDP compared to miglitol and miglustat
    Sarah F Jenkinson
    Chemistry Research Laboratory, Department of Chemistry, University of Oxford, Mansfield Road, Oxford OX1 3TA, UK
    J Org Chem 78:7380-97. 2013
    ..The partial rescue of the defective F508del-CFTR function in CF-KM4 cells by L-isoDMDP is compared with miglustat and isoLAB in an approach to the treatment of cystic fibrosis...
  26. ncbi request reprint Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling
    David C A Neville
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Anal Biochem 331:275-82. 2004
    ..This new approach may also be used to analyze both N- and O-linked oligosaccharides...
  27. pmc Substrate reduction therapy in mouse models of the glycosphingolipidoses
    Frances M Platt
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Philos Trans R Soc Lond B Biol Sci 358:947-54. 2003
    ..A second drug, N-butyldeoxyglactonojirimycin, looks very promising for treating storage diseases with neurological involvement as high systemic dosing is achievable without any side-effects...
  28. ncbi request reprint Restricted processing of glycans by endomannosidase in mammalian cells
    Nikolay V Kukushkin
    Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, UK
    Glycobiology 22:1282-8. 2012
    ....
  29. ncbi request reprint Synthesis of the enantiomers of XYLNAc and LYXNAc: comparison of β-N-acetylhexosaminidase inhibition by the 8 stereoisomers of 2-N-acetylamino-1,2,4-trideoxy-1,4-iminopentitols
    Elizabeth V Crabtree
    Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Org Biomol Chem 12:3932-43. 2014
    ..Both XYLNAc and LABNAc are potent inhibitors against HexNAcases. None of the compounds show any inhibition of α-GalNAcase...
  30. ncbi request reprint Inhibition of glycogen breakdown by imino sugars in vitro and in vivo
    Ulrika Andersson
    Department of Biochemistry, Glycobiology Institute, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem Pharmacol 67:697-705. 2004
    ..Depending on the dose of these compounds used, there is the potential for glycogen catabolism to be partially impaired in experimental animals and man...
  31. ncbi request reprint Synthesis from D-altrose of (5R,6R,7R,8S)-5,7-dihydroxy-8-hydroxymethylconidine and 2,4-dideoxy-2,4-imino-D-glucitol, azetidine analogues of swainsonine and 1,4-dideoxy-1,4-imino-D-mannitol
    Noelia Araujo
    Chemistry Research Laboratory, Department of Chemistry, University of Oxford, Mansfield Road, Oxford, OX1 3TA, UK
    Org Lett 14:4174-7. 2012
    ..Ring closure of a 3,5-di-O-triflate derived from D-altrose with benzylamine allowed the formation of both monocyclic and bicyclic azetidine analogues of swainsonine...
  32. ncbi request reprint Targeting glycosylation as a therapeutic approach
    Raymond A Dwek
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Nat Rev Drug Discov 1:65-75. 2002
    ..Two such approaches that use imino sugars to affect glycosylation enzymes now show considerable promise in the treatment of viral infections, such as hepatitis B, and glucosphingolipid storage disorders, such as Gaucher disease...
  33. ncbi request reprint Looking glass inhibitors: efficient synthesis and biological evaluation of D-deoxyfuconojirimycin
    Yves Blériot
    Chemistry Research Laboratory, Department of Chemistry, University of Oxford, Mansfield Road, Oxford OX1 3TA, UK
    Carbohydr Res 340:2713-8. 2005
    ..1,6-Dideoxygalactostatin, the mirror image of 1-deoxy-L-fuconojirimycin, was efficiently prepared from 2,3-O-isopropylidene-L-lyxonolactone in four steps and evaluated as a glycosidase inhibitor...
  34. pmc Small-molecule therapeutics for the treatment of glycolipid lysosomal storage disorders
    Terry D Butters
    Glycobiology Institute, Department of Biochemistry, University of Oxford, Oxford OX1 3QU, UK
    Philos Trans R Soc Lond B Biol Sci 358:927-45. 2003
    ..The implications of these studies and the prospects of improvement to the design of iminosugar compounds for treating Gaucher and other GSL lysosomal storage disorders will be discussed...
  35. ncbi request reprint New developments in treating glycosphingolipid storage diseases
    Frances M Platt
    Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Adv Exp Med Biol 564:117-26. 2005
  36. doi request reprint Development of a single column method for the separation of lipid- and protein-derived oligosaccharides
    David C A Neville
    Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    J Proteome Res 8:681-7. 2009
    ..Therefore, analysis of HILIC- or HIAX-separated fluorophore-labeled oligosaccharides can be performed using a single HPLC system with a single set of eluents following a simple column change...
  37. doi request reprint Probing replacement of pyrophosphate via click chemistry; synthesis of UDP-sugar analogues as potential glycosyl transferase inhibitors
    Kar Kheng Yeoh
    Department of Chemistry, Chemistry Research Laboratory, University of Oxford, Mansfield Road, Oxford OX1 3TA, UK
    Carbohydr Res 344:586-91. 2009
    ..None of the compounds accessed displayed significant inhibitory activity at concentrations of up to 4.5mM in an assay against bovine milk beta-1,4-galactosyltransferase...
  38. doi request reprint Glycosphingolipid disorders of the brain
    Stephanie D Boomkamp
    Glycobiology Institute, Department of Biochemistry, University of Oxford, OX1 3QU, Oxford, UK
    Subcell Biochem 49:441-67. 2008
    ..However, recent advances in enzyme replacement, bone marrow transplantation, gene transfer, substrate reduction and chaperon-mediated therapy provide great potential in treating these devastating disorders...
  39. ncbi request reprint Increased glycosphingolipid levels in serum and aortae of apolipoprotein E gene knockout mice
    Brett Garner
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    J Lipid Res 43:205-14. 2002
    ..The apoE-/- mouse therefore represents a useful model to study the potential role of GSL metabolism in atherogenesis...
  40. pmc Preparation, biochemical characterization and biological properties of radiolabelled N-alkylated deoxynojirimycins
    Howard R Mellor
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, U K
    Biochem J 366:225-33. 2002
    ..Overall these data provide further definition of the molecular features of alkylated imino sugars that influence tissue selectivity and efficacy for cellular enzyme inhibition...
  41. ncbi request reprint Glucosylceramide modulates membrane traffic along the endocytic pathway
    Dan J Sillence
    Glycobiology Institute, Department of Biochemistry, South Parks Road, University of Oxford, Oxford OX1 3QU, UK
    J Lipid Res 43:1837-45. 2002
    ..These data demonstrate that both increases and decreases in glucosylceramide levels can dramatically alter the endocytic targeting of lactosylceramide and suggest a role for glucosylceramide in regulation of membrane transport...
  42. pmc Reversible infertility in male mice after oral administration of alkylated imino sugars: a nonhormonal approach to male contraception
    Aarnoud C van der Spoel
    The Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, United Kingdom
    Proc Natl Acad Sci U S A 99:17173-8. 2002
    ..These compounds therefore may be new leads in the development of a male contraceptive, especially because NB-DNJ has already been through extensive evaluation in various mammals, including man...
  43. ncbi request reprint Therapeutic applications of imino sugars in lysosomal storage disorders
    Terry D Butters
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, UK
    Curr Top Med Chem 3:561-74. 2003
    ..One imino sugar, N-butyl-DNJ (NB-DNJ) has been in clinical trials for type 1 Gaucher disease and has shown to be an effective therapy for this disorder...
  44. doi request reprint Scalable syntheses of both enantiomers of DNJNAc and DGJNAc from glucuronolactone: the effect of N-alkylation on hexosaminidase inhibition
    Andreas F G Glawar
    Oxford Glycobiology Institute, University of Oxford, South Parks Road, Oxford, OX1 3QU, UK
    Chemistry 18:9341-59. 2012
    ....
  45. doi request reprint Hydrophilic interaction liquid chromatography of anthranilic acid-labelled oligosaccharides with a 4-aminobenzoic acid ethyl ester-labelled dextran hydrolysate internal standard
    David C A Neville
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, United Kingdom
    J Chromatogr A 1233:66-70. 2012
    ..Therefore, this paper provides the first method for determination of HPLC-derived GU values of fluorescently labelled oligosaccharides using an internal calibrant...
  46. ncbi request reprint Storage solutions: treating lysosomal disorders of the brain
    Mylvaganam Jeyakumar
    Department of Biochemistry, University of Oxford, UK
    Nat Rev Neurosci 6:713-25. 2005
    ..We review the developments in this field and discuss the similarities in pathological features between these diseases and some more common neurodegenerative disorders...
  47. ncbi request reprint Synthesis of fluorescence-labelled disaccharide substrates of glucosidase II
    Ian Cumpstey
    Dyson Perrins Laboratory, Oxford University, South Parks Road, Oxford OX1 3QY, UK
    Carbohydr Res 338:1937-49. 2003
    ..Selective activation of a fully armed thioglycoside in the presence of n-pentenyl glycosides was readily achieved by the use of methyl triflate as promoter...
  48. ncbi request reprint Therapeutic targets for inhibitors of glycosylation
    Dominic S Alonzi
    Oxford Glycobiology Institute, Department of Biochemistry, Oxford University, South Parks Road, Oxford OX1 3QU, UK
    Chimia (Aarau) 65:35-9. 2011
    ....
  49. pmc Adduction of cholesterol 5,6-secosterol aldehyde to membrane-bound myelin basic protein exposes an immunodominant epitope
    Natalie K Cygan
    The Scripps Oxford Laboratory, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochemistry 50:2092-100. 2011
    ....
  50. doi request reprint Urinary glycan markers for disease
    Dominic S Alonzi
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, UK
    Biochem Soc Trans 39:393-8. 2011
    ..The ability to predict disease status in microlitre amounts of readily available non-invasive urine samples indicates that rapid methods for screening can be developed...
  51. ncbi request reprint Modulation of THP-1 macrophage and cholesterol-loaded foam cell apolipoprotein E levels by glycosphingolipids
    Brett Garner
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford, OX1 3QU, United Kingdom
    Biochem Biophys Res Commun 290:1361-7. 2002
    ..Furthermore, the efflux of glycosphingolipids from macrophage foam cells to HDL could indicate a potential pathway for their removal from the artery wall and subsequent delivery to the liver...
  52. ncbi request reprint An inducible mouse model of late onset Tay-Sachs disease
    Mylvaganam Jeyakumar
    Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford OX1 3QU, United Kingdom
    Neurobiol Dis 10:201-10. 2002
    ..Repeat breeding of a large cohort of female Tay-Sachs mice confirmed that pregnancy induces late onset Tay-Sachs disease. Onset of symptoms correlated with reduced up-regulation of hexosaminidase B, a component of the bypass pathway...
  53. ncbi request reprint Control in the N-linked glycoprotein biosynthesis pathway
    Terry D Butters
    Glycobiology Institute, University of Oxford, South Parks Road, OX1 3QU, Oxford, United Kingdom
    Chem Biol 9:1266-8. 2002
    ..The individual steps of this complex process are slowly being elucidated. In this issue, the Imperiali group further dissects the mechanics of oligosaccharyl transferase using substrate analogs...
  54. pmc Non-specific accumulation of glycosphingolipids in GNE myopathy
    Katherine A Patzel
    Oxford Glycobiology Institute, Department of Biochemistry, University of Oxford, Oxford, OX1 3QU, UK
    J Inherit Metab Dis 37:297-308. 2014
    ..The connection between the impairment of sialic acid synthesis and muscle pathology in GNE myopathy remains poorly understood...
  55. ncbi request reprint Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease
    Boris Brumshtein
    Department of Structural Biology, Weizmann Institute of Science, Rehovot 76100, Israel
    J Biol Chem 282:29052-8. 2007
    ..Together, these results have significance for understanding the mechanism of action of GlcCerase and the mode of GlcCerase chaperoning by imino sugars...
  56. doi request reprint New synthetic seven-membered 1-azasugars displaying potent inhibition towards glycosidases and glucosylceramide transferase
    Hongqing Li
    UMR 7611, Laboratoire de Synthese Organique, Institut de Chimie Moleculaire FR 2769, Universite Pierre et Marie Curie Paris 6, 75005 Paris, France
    Chembiochem 9:253-60. 2008
    ....
  57. ncbi request reprint Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C
    Robin H Lachmann
    Department of Medicine, University of Cambridge, Cambridge CB2 2QQ, UK
    Neurobiol Dis 16:654-8. 2004
    ..These observations support the use of SRT in patients with this devastating neurodegenerative disease...
  58. pmc Gangliosides play an important role in the organization of CD82-enriched microdomains
    Elena Odintsova
    Cancer Research U K Institute for Cancer Studies, The University of Birmingham, Edgbaston, Birmingham B15 2TT, UK
    Biochem J 400:315-25. 2006
    ..Furthermore, these results demonstrate that the association between different tetraspanins in TERM is controlled by distinct mechanisms and identify Neu3 as a first physiological regulator of the integrity of these microdomains...
  59. ncbi request reprint 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
    Ryota Suganuma
    Institute for Biogenesis Research, University of Hawaii School of Medicine, Honolulu, Hawaii 96822, USA
    Biol Reprod 72:805-13. 2005
    ..This indicates that during short-term administration, alkylated imino sugars alter sperm morphology and physiology but do not diminish the genetic potential of spermatozoa...
  60. ncbi request reprint Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase
    Michael A Simpson
    Department of Medical Genetics, St George s Hospital Medical School, University of London, Cranmer Terrace, London SW17 0RE, UK
    Nat Genet 36:1225-9. 2004
    ....
  61. ncbi request reprint Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease
    Jean Pyo Lee
    Stem Cell and Regeneration Program, Center for Neuroscience and Aging Research, Burnham Institute for Medical Research, La Jolla, California 92037, USA
    Nat Med 13:439-47. 2007
    ..Appreciating that NSCs exhibit a broad repertoire of potentially therapeutic actions, of which neuronal replacement is but one, may help in formulating rational multimodal strategies for the treatment of neurodegenerative diseases...
  62. pmc Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases
    Stephan D Gadola
    Weatherall Institute of Molecular Medicine, Tumour Immunology Group, John Radcliffe Hospital, Oxford OX3 9DS, England, UK
    J Exp Med 203:2293-303. 2006
    ..These data suggest that GSL storage may result in alterations in thymic selection of iNKT cells caused by impaired presentation of selecting ligands...
  63. ncbi request reprint Characterization of the oligosaccharide component of microsomal beta-glucuronidase from rat liver
    Dorota Hoja-Łukowicz
    Department of Animal Physiology, Institute of Zoology, Jagiellonian University, 6 Ingardena Street, 30 060 Krakow, Poland
    Biochimie 86:363-72. 2004
    ..The presence of O-linked glycans and branched N-linked glycans in a microsomal enzyme, in relation to the current view of glycosyltransferase compartmentalization in the Golgi is discussed...
  64. pmc Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals
    Anneliese O Speak
    Department of Pharmacology, University of Oxford, Mansfield Road, Oxford OX1 3QT, United Kingdom
    Proc Natl Acad Sci U S A 104:5971-6. 2007
    ..iGb3 is therefore unlikely to be a physiologically relevant iNKT cell-selecting ligand in mouse and humans. A detailed study is now warranted to better understand the nature of iNKT cell-selecting ligand(s) in vivo...
  65. ncbi request reprint Human invariant NKT cells display alloreactivity instructed by invariant TCR-CD1d interaction and killer Ig receptors
    Scott Patterson
    Department of Hematology, Imperial College London, Hammersmith Hospital Campus, London, United Kingdom
    J Immunol 181:3268-76. 2008
    ..Thus, iNKT cells can display alloreactivity, for which they use mechanisms characteristic of both NK and conventional T cells...