8P41
 
 | | Crystal structure of glucocerebrosidase in complex with allosteric activator | | Descriptor: | 2-[2-[[3,5-bis(trifluoromethyl)phenyl]methylsulfanyl]ethanoylamino]-5-chloranyl-benzoic acid, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | | Authors: | Schulze, M.-S. | | Deposit date: | 2023-05-18 | | Release date: | 2024-03-06 | | Last modified: | 2024-05-01 | | Method: | X-RAY DIFFRACTION (1.83 Å) | | Cite: | Identification of ss-Glucocerebrosidase Activators for Glucosylceramide hydrolysis.
Chemmedchem, 19, 2024
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3GXI
 | | Crystal structure of acid-beta-glucosidase at pH 5.5 | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, PHOSPHATE ION | | Authors: | Lieberman, R.L. | | Deposit date: | 2009-04-02 | | Release date: | 2009-05-05 | | Last modified: | 2020-07-29 | | Method: | X-RAY DIFFRACTION (1.84 Å) | | Cite: | Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
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6T13
 | | CRYSTAL STRUCTURE OF GLUCOCEREBROSIDASE IN COMPLEX WITH A PYRROLOPYRAZINE | | Descriptor: | 1,2-ETHANEDIOL, 1-[4-[2-(4-methoxyphenyl)-5-methyl-pyrrolo[2,3-b]pyrazin-6-yl]piperidin-1-yl]ethanone, 2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, ... | | Authors: | Benz, J, Ehler, A, Hug, M, Huber, S, Rufer, A.C, Guba, W, Jagasia, R, Hofmann, E.C, Rodriguez Sarmiento, R.M. | | Deposit date: | 2019-10-03 | | Release date: | 2020-12-23 | | Last modified: | 2024-01-24 | | Method: | X-RAY DIFFRACTION (1.85 Å) | | Cite: | Novel beta-Glucocerebrosidase Activators That Bind to a New Pocket at a Dimer Interface and Induce Dimerization.
Angew.Chem.Int.Ed.Engl., 60, 2021
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7NWV
 | | Structure of recombinant human beta-glucocerebrosidase in complex with BODIPY Tagged Cyclophellitol activity based probe | | Descriptor: | (1~{S},2~{R},3~{R},4~{S},5~{S})-4-[[4-[4-[2,2-bis(fluoranyl)-4,6,10,12-tetramethyl-3-aza-1-azonia-2-boranuidatricyclo[7.3.0.0^{3,7}]dodeca-1(12),4,6,8,10-pentaen-8-yl]butyl]-1,2,3-triazol-1-yl]methyl]cyclohexane-1,2,3,5-tetrol, 1,2-ETHANEDIOL, 1-deoxy-alpha-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | | Authors: | Rowland, R.J, Davies, G.J. | | Deposit date: | 2021-03-17 | | Release date: | 2022-03-30 | | Last modified: | 2024-01-31 | | Method: | X-RAY DIFFRACTION (1.86 Å) | | Cite: | Fluorescence polarisation activity-based protein profiling for the identification of deoxynojirimycin-type inhibitors selective for lysosomal retaining alpha- and beta-glucosidases.
Chem Sci, 14, 2023
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6Q6K
 | | Crystal structure of recombinant human beta-glucocerebrosidase in complex with cyclophellitol activity based probe with Cy5 tag (ME569) | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, 6-[3,3-dimethyl-2-[(1~{E},3~{E},5~{E})-5-(1,3,3-trimethylindol-2-ylidene)penta-1,3-dienyl]indol-1-ium-1-yl]-~{N}-[[1-[[(1~{S},2~{R},3~{R},4~{S},6~{S})-2,3,4,6-tetrakis(oxidanyl)cyclohexyl]methyl]-1,2,3-triazol-4-yl]methyl]hexanamide, ... | | Authors: | Rowland, R.J, Davies, G.J. | | Deposit date: | 2018-12-11 | | Release date: | 2019-03-27 | | Last modified: | 2024-01-24 | | Method: | X-RAY DIFFRACTION (1.92 Å) | | Cite: | Functionalized Cyclophellitols Are Selective Glucocerebrosidase Inhibitors and Induce a Bona Fide Neuropathic Gaucher Model in Zebrafish.
J.Am.Chem.Soc., 141, 2019
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2V3F
 | | acid-beta-glucosidase produced in carrot | | Descriptor: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, GLUCOSYLCERAMIDASE, SULFATE ION, ... | | Authors: | Shaaltiel, Y, Bartfeld, D, Hashmueli, S, Baum, G, Brill-Almon, E, Galili, G, Dym, O, Boldin-Adamsky, S.A, Silman, I, Sussman, J.L, Futerman, A.H, Aviezer, D, Israel Structural Proteomics Center (ISPC) | | Deposit date: | 2007-06-17 | | Release date: | 2008-04-08 | | Last modified: | 2023-12-13 | | Method: | X-RAY DIFFRACTION (1.95 Å) | | Cite: | Production of Glucocerebrosidase with Terminal Mannose Glycans for Enzyme Replacement Therapy of Gaucher'S Disease Using a Plant Cell System.
Plant Biotechnol.J., 5, 2007
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2V3D
 | | acid-beta-glucosidase with N-butyl-deoxynojirimycin | | Descriptor: | (2R,3R,4R,5S)-1-BUTYL-2-(HYDROXYMETHYL)PIPERIDINE-3,4,5-TRIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, GLUCOSYLCERAMIDASE, ... | | Authors: | Brumshtein, B, Greenblatt, H.M, Butters, T.D, Shaaltiel, Y, Aviezer, D, Silman, I, Futerman, A.H, Sussman, J.L. | | Deposit date: | 2007-06-17 | | Release date: | 2007-08-14 | | Last modified: | 2023-12-13 | | Method: | X-RAY DIFFRACTION (1.96 Å) | | Cite: | 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.
J.Biol.Chem., 282, 2007
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2V3E
 | | acid-beta-glucosidase with N-nonyl-deoxynojirimycin | | Descriptor: | (2R,3R,4R,5S)-2-(HYDROXYMETHYL)-1-NONYLPIPERIDINE-3,4,5-TRIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, GLUCOSYLCERAMIDASE, ... | | Authors: | Brumshtein, B, Greenblatt, H.M, Butters, T.D, Shaaltiel, Y, Aviezer, D, Silman, I, Futerman, A.H, Sussman, J.L. | | Deposit date: | 2007-06-17 | | Release date: | 2007-08-21 | | Last modified: | 2023-12-13 | | Method: | X-RAY DIFFRACTION (2 Å) | | Cite: | 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.
J.Biol.Chem., 282, 2007
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1OGS
 | | human acid-beta-glucosidase | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, ... | | Authors: | Dvir, H, Harel, M, McCarthy, A.A, Toker, L, Silman, I, Futerman, A.H, Sussman, J.L. | | Deposit date: | 2003-05-13 | | Release date: | 2003-07-03 | | Last modified: | 2020-07-29 | | Method: | X-RAY DIFFRACTION (2 Å) | | Cite: | X-Ray Structure of Human Acid-Beta-Glucosidase, the Defective Enzyme in Gaucher Disease
Embo Rep., 4, 2003
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6MOZ
 | | Structure of acid-beta-glucosidase in complex with an aromatic pyrrolidine iminosugar inhibitor | | Descriptor: | (2R,3S,4R)-2-{[4-(3,5-dichlorophenyl)-1H-1,2,3-triazol-1-yl]methyl}pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, GLYCEROL, ... | | Authors: | Patterson-Orazem, A.C, Lieberman, R.L. | | Deposit date: | 2018-10-05 | | Release date: | 2019-06-19 | | Last modified: | 2023-10-11 | | Method: | X-RAY DIFFRACTION (2.1 Å) | | Cite: | Exploring substituent diversity on pyrrolidine-aryltriazole iminosugars: Structural basis of beta-glucocerebrosidase inhibition.
Bioorg.Chem., 86, 2019
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2NSX
 | | Structure of acid-beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 5-HYDROXYMETHYL-3,4-DIHYDROXYPIPERIDINE, GLYCEROL, ... | | Authors: | Lieberman, R.L, Petsko, G.A, Ringe, D. | | Deposit date: | 2006-11-06 | | Release date: | 2006-12-26 | | Last modified: | 2023-08-30 | | Method: | X-RAY DIFFRACTION (2.11 Å) | | Cite: | Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease.
Nat.Chem.Biol., 3, 2007
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2VT0
 | | X-ray structure of a conjugate with conduritol-beta-epoxide of acid-beta-glucosidase overexpressed in cultured plant cells | | Descriptor: | (1R,2R,3S,4S,5S,6S)-CYCLOHEXANE-1,2,3,4,5,6-HEXOL, GLUCOSYLCERAMIDASE, SULFATE ION, ... | | Authors: | Brumshtein, B, Greenblatt, H.M, Shaaltiel, Y, Aviezer, D, Silman, I, Futerman, A.H, Sussman, J.L. | | Deposit date: | 2008-05-03 | | Release date: | 2008-09-23 | | Last modified: | 2023-12-13 | | Method: | X-RAY DIFFRACTION (2.15 Å) | | Cite: | Acid Beta-Glucosidase: Insights from Structural Analysis and Relevance to Gaucher Disease Therapy.
Biol.Chem., 389, 2008
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5LVX
 | | Crystal structure of glucocerebrosidase with an inhibitory quinazoline modulator | | Descriptor: | 11-[(2~{R})-2-[(2-pyridin-3-ylquinazolin-4-yl)amino]-2,3-dihydro-1~{H}-inden-5-yl]undec-10-ynoic acid, 11-[(2~{S})-2-[(2-pyridin-3-ylquinazolin-4-yl)amino]-2,3-dihydro-1~{H}-inden-5-yl]undec-10-ynoic acid, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | | Authors: | Zheng, J, Chen, L, Skinner, O.S, Lansbury, P, Skerlj, R, Mrosek, M, Heunisch, U, Krapp, S, Weigand, S, Charrow, J, Schwake, M, Kelleher, N.L, Silverman, R.B, Krainc, D. | | Deposit date: | 2016-09-14 | | Release date: | 2017-10-25 | | Last modified: | 2020-07-29 | | Method: | X-RAY DIFFRACTION (2.2 Å) | | Cite: | beta-Glucocerebrosidase Modulators Promote Dimerization of beta-Glucocerebrosidase and Reveal an Allosteric Binding Site.
J. Am. Chem. Soc., 140, 2018
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3GXM
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9F9Z
 | | Gcase in complex with small molecule inhibitor 1 | | Descriptor: | (2~{S})-1-(2,6-dimethylphenoxy)propan-2-amine, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Lysosomal acid glucosylceramidase, ... | | Authors: | Tisi, D, Cleasby, A. | | Deposit date: | 2024-05-09 | | Release date: | 2024-07-03 | | Method: | X-RAY DIFFRACTION (2.279 Å) | | Cite: | Fragment-Based Discovery of a Series of Allosteric-Binding Site Modulators of beta-Glucocerebrosidase.
J.Med.Chem., 2024
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2WCG
 | | X-ray structure of acid-beta-glucosidase with N-octyl(cyclic guanidine)-nojirimycin in the active site | | Descriptor: | CHLORIDE ION, GLUCOSYLCERAMIDASE, N-[(3E,5R,6R,7S,8R,8AR)-5,6,7,8-TETRAHYDROXYHEXAHYDROIMIDAZO[1,5-A]PYRIDIN-3(2H)-YLIDENE]OCTAN-1-AMINIUM, ... | | Authors: | Brumshtein, B, Aguilar, M, Garcia-Moreno, M.I, Mellet, C.O, Garcia-Fernandez, J.M, Silman, I, Shaaltiel, Y, Aviezer, D, Sussman, J.L, Futerman, A.H. | | Deposit date: | 2009-03-12 | | Release date: | 2009-11-24 | | Last modified: | 2023-12-13 | | Method: | X-RAY DIFFRACTION (2.3 Å) | | Cite: | 6-Amino-6-Deoxy-5,6-Di-N-(N'-Octyliminomethylidene)Nojirimycin: Synthesis, Biological Evaluation, and Crystal Structure in Complex with Acid Beta-Glucosidase.
Chembiochem, 10, 2009
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2NT1
 | | Structure of acid-beta-glucosidase at neutral pH | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, PHOSPHATE ION | | Authors: | Lieberman, R.L, Petsko, G.A, Ringe, D. | | Deposit date: | 2006-11-06 | | Release date: | 2006-12-26 | | Last modified: | 2023-08-30 | | Method: | X-RAY DIFFRACTION (2.3 Å) | | Cite: | Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease.
Nat.Chem.Biol., 3, 2007
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2XWE
 | | X-RAY STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH 5N,6S-(N'-(N-OCTYL)IMINO)-6-THIONOJIRIMYCIN IN THE ACTIVE SITE | | Descriptor: | (3Z,5S,6R,7S,8R,8aS)-3-(octylimino)hexahydro[1,3]thiazolo[3,4-a]pyridine-5,6,7,8-tetrol, GLUCOSYLCERAMIDASE, PHOSPHATE ION, ... | | Authors: | Brumshtein, B, Aguilar-Moncayo, M, Benito, J.M, Ortiz Mellet, C, Garcia Fernandez, J.M, Silman, I, Shaaltiel, Y, Sussman, J.L, Futerman, A.H. | | Deposit date: | 2010-11-02 | | Release date: | 2011-09-14 | | Last modified: | 2023-12-20 | | Method: | X-RAY DIFFRACTION (2.31 Å) | | Cite: | Cyclodextrin-Mediated Crystallization of Acid Beta-Glucosidase in Complex with Amphiphilic Bicyclic Nojirimycin Analogues.
Org.Biomol.Chem., 9, 2011
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1Y7V
 | | X-ray structure of human acid-beta-glucosidase covalently bound to conduritol B epoxide | | Descriptor: | 1,2,3,4,5,6-HEXAHYDROXY-CYCLOHEXANE, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, ... | | Authors: | Premkumar, L, Sawkar, A.R, Boldin-Adamsky, S, Toker, L, Silman, I, Kelly, J.W, Futerman, A.H, Sussman, J.L, Israel Structural Proteomics Center (ISPC) | | Deposit date: | 2004-12-10 | | Release date: | 2005-04-12 | | Last modified: | 2023-08-23 | | Method: | X-RAY DIFFRACTION (2.4 Å) | | Cite: | X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease.
J.Biol.Chem., 280, 2005
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3RIL
 | | The acid beta-glucosidase active site exhibits plasticity in binding 3,4,5,6-tetrahydroxyazepane-based inhibitors: implications for pharmacological chaperone design for gaucher disease | | Descriptor: | (3S,4R,5R,6S)-azepane-3,4,5,6-tetrol, 2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, ... | | Authors: | Orwig, S.D, Lieberman, R.L. | | Deposit date: | 2011-04-13 | | Release date: | 2012-03-14 | | Last modified: | 2020-07-29 | | Method: | X-RAY DIFFRACTION (2.4 Å) | | Cite: | Binding of 3,4,5,6-tetrahydroxyazepanes to the acid-beta-glucosidase active site: implications for pharmacological chaperone design for Gaucher disease
Biochemistry, 50, 2011
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3GXF
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3RIK
 | | The acid beta-glucosidase active site exhibits plasticity in binding 3,4,5,6-tetrahydroxyazepane-based inhibitors: implications for pharmacological chaperone design for gaucher disease | | Descriptor: | (3S,4R,5R,6S)-1-(2-hydroxyethyl)azepane-3,4,5,6-tetrol, 2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, ... | | Authors: | Orwig, S.D, Lieberman, R.L. | | Deposit date: | 2011-04-13 | | Release date: | 2012-03-14 | | Last modified: | 2020-07-29 | | Method: | X-RAY DIFFRACTION (2.48 Å) | | Cite: | Binding of 3,4,5,6-tetrahydroxyazepanes to the acid-beta-glucosidase active site: implications for pharmacological chaperone design for Gaucher disease
Biochemistry, 50, 2011
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2F61
 | | Crystal structure of partially deglycosylated acid beta-glucosidase | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Acid beta-glucosidase, ... | | Authors: | Hegde, R.S, Grabowski, G. | | Deposit date: | 2005-11-28 | | Release date: | 2005-12-27 | | Last modified: | 2023-08-23 | | Method: | X-RAY DIFFRACTION (2.5 Å) | | Cite: | Analyses of Variant Acid beta-Glucosidases: EFFECTS OF GAUCHER DISEASE MUTATIONS.
J.Biol.Chem., 281, 2006
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3GXD
 | | Crystal structure of Apo acid-beta-glucosidase pH 4.5 | | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, Glucosylceramidase, PHOSPHATE ION | | Authors: | Lieberman, R.L. | | Deposit date: | 2009-04-02 | | Release date: | 2009-05-05 | | Last modified: | 2023-09-06 | | Method: | X-RAY DIFFRACTION (2.5 Å) | | Cite: | Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
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6Q1N
 | | Glucocerebrosidase in complex with pharmacological chaperone IMX8 | | Descriptor: | (2R,3S,4S,5R)-2-[2-(methylsulfanyl)ethyl]piperidine-3,4,5-triol, 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | | Authors: | Vickers, C, Withers, S.G, Boraston, A.B. | | Deposit date: | 2019-08-05 | | Release date: | 2020-08-19 | | Method: | X-RAY DIFFRACTION (2.526 Å) | | Cite: | Pharmacological chaperones for GCase that switch conformation with pH enhance enzyme levels in Gaucher animal models.
To Be Published
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