Search Results - "Dwek, R. A"

Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis by 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.

“…Mouse models of the GM2 gangliosidoses [Tay‐Sachs, late onset Tay‐Sachs (LOTS), Sandhoff] and GM1 gangliosidosis have been studied to determine whether there…”
Get full text

Sustained therapeutic effects of oral miglustat (Zavesca, N‐butyldeoxynojirimycin, OGT 918) in type I Gaucher disease by 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.

“…It has been shown that treatment with miglustat (Zavesca, N‐butyldeoxynojirimycin, OGT 918) improves key clinical features of type I Gaucher disease after 1…”
Get full text

Glycosylation and the Immune System by Rudd, Pauline M., Elliott, Tim, Cresswell, Peter, Wilson, Ian A., Dwek, Raymond A.

“…Almost all of the key molecules involved in the innate and adaptive immune response are glycoproteins. In the cellular immune system, specific glycoforms are…”
Get full text

Crystal Structure of a Neutralizing Human IgG against HIV-1: A Template for Vaccine Design by Saphire, Erica Ollmann, Paul W. H. I. Parren, Pantophlet, Ralph, Zwick, Michael B., Morris, Garrett M., Rudd, Pauline M., Dwek, Raymond A., Stanfield, Robyn L., Burton, Dennis R., Wilson, Ian A.

“…We present the crystal structure at 2.7 angstrom resolution of the human antibody IgG1 b12. Antibody b12 recognizes the CD4-binding site of human…”
Get full text

Complement Regulation at the Molecular Level: The Structure of Decay-Accelerating Factor by Lukacik, P., Roversi, P., White, J., Esser, D., Smith, G. P., Billington, J., Williams, P. A., Rudd, P. M., Wormald, M. R., Harvey, D. J., Crispin, M. D. M., Radcliffe, C. M., Dwek, R. A., Evans, D. J., Morgan, B. P., Smith, R. A. G., Lea, S. M.

“…The human complement regulator CD55 is a key molecule protecting self-cells from complement-mediated lysis. X-ray diffraction and analytical…”
Get full text

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis by Jeyakumar, M., Butters, T. D., Dwek, R. A., Platt, F. M.

“…Paediatric neurodegenerative diseases are frequently caused by inborn errors in glycosphingolipid (GSL) catabolism and are collectively termed the…”
Get full text

Glycobiology: "towards understanding the function of sugars" by Dwek, R A

Get more information

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis by PLATT, F. M, NEISES, G. R, DWEK, R. A, BUTTERS, T. D

“…The imino sugar deoxynojirimycin and its alkylated derivatives are inhibitors of the N-linked oligosaccharide processing enzymes alpha-glucosidase I and II…”
Get full text

Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N‐butyldeoxynojirimycin treatment by Heare, T., Alp, N. J., Priestman, D. A., Kulkarni, A. B., Qasba, P., Butters, T. D., Dwek, R. A., Clarke, K., Channon, K. M., Platt, F. M.

“…Summary Objective: Fabry disease results from α‐gala‐ ctosidase A deficiency and is characterized by the lysosomal accumulation of globotriaosylceramide…”
Get full text

Glycosylation: heterogeneity and the 3D structure of proteins by Rudd, P M, Dwek, R A

“…Glycoproteins generally exist as populations of glycosylated variants (glycoforms) of a single polypeptide. Although the same glycosylation machinery is…”
Get more information

N-butyldeoxynojirimycin causes weight loss as a result of appetite suppression in lean and obese mice by Priestman, D.A, van der Spoel, A.C, Butters, T.D, Dwek, R.A, Platt, F.M

“…To determine the mechanism of weight loss caused by high doses of N-butyldeoxynojirimycin (NB-DNJ) in healthy lean and leptin-deficient obese (ob/ob) mice…”
Get full text

A statistical analysis of N- and O-glycan linkage conformations from crystallographic data by Petrescu, Andrei J., Petrescu, Stefana M., Dwek, Raymond A., Wormald, Mark R.

“…We have generated a database of 639 glycosidic linkage structures by an exhaustive survey of the available crystallographic data for isolated oligosaccharides,…”
Get full text

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

“…Article Usage Stats Services JVI Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
Get full text

Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy by Platt, F. M., Jeyakumar, M., Andersson, U., Priestman, D. A., Dwek, R. A., Butters, T. D., Cox, T. M., Lachmann, R. H., Hollak, C., Aerts, J. M. F. G., Van Weely, S., Hrebícek, M., Moyses, C., Gow, I., Elstein, D., Zimran, A.

“…The glycosphingolipid (GSL) lysosomal storage diseases are caused by mutations in the genes encoding the glycohydrolases that catabolize GSLs within lysosomes…”
Get full text

Glycobiology by Rademacher, T W, Parekh, R B, Dwek, R A

Get full text

Prevention of Lysosomal Storage in Tay-Sachs Mice Treated with N-Butyldeoxynojirimycin by 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.

“…The glycosphingolipid (GSL) lysosomal storage diseases result from the inheritance of defects in the genes encoding the enzymes required for catabolism of GSLs…”
Get full text

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

“…N-Butyldeoxynojirimycin ( NB-DNJ) inhibits the ceramide glucosyltransferase which catalyses the first step in glycosphingolipid (GSL) biosynthesis. It has the…”
Get full text

Prion Glycoprotein:  Structure, Dynamics, and Roles for the Sugars by Rudd, Pauline M, Wormald, Mark R, Wing, David R, Prusiner, Stanley B, Dwek, Raymond A

“…The prion protein contains two N-linked glycosylation sites and a glycosylphosphatidylinositol (GPI) anchor. The large size of the N-linked sugars, together…”
Get full text

The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding by Fischer, P B, Collin, M, Karlsson, G B, James, W, Butters, T D, Davis, S J, Gordon, S, Dwek, R A, Platt, F M

“…Article Usage Stats Services JVI Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
Get full text

N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120 by Fischer, P B, Karlsson, G B, Butters, T D, Dwek, R A, Platt, F M

“…Article Usage Stats Services JVI Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
Get full text