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5LVX
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CRYSTAL STRUCTURE OF GLUCOCEREBROSIDASE WITH AN INHIBITORY QUINAZOLINE MODULATOR
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, BETA-D-MANNOSE, ...
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:2018-05-23
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
1OGS
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HUMAN ACID-BETA-GLUCOSIDASE
Descriptor:GLUCOSYLCERAMIDASE, SULFATE ION, N-ACETYL-D-GLUCOSAMINE
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:2012-01-25
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
1Y7V
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X-RAY STRUCTURE OF HUMAN ACID-BETA-GLUCOSIDASE COVALENTLY BOUND TO CONDURITOL B EPOXIDE
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, SULFATE ION, ...
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:2011-07-13
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
2F61
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CRYSTAL STRUCTURE OF PARTIALLY DEGLYCOSYLATED ACID BETA-GLUCOSIDASE
Descriptor:Acid beta-glucosidase, 2-(ACETYLAMINO)-2-DEOXY-A-D-GLUCOPYRANOSE, SULFATE ION, ...
Authors:Hegde, R.S., Grabowski, G.
Deposit date:2005-11-28
Release date:2005-12-27
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.5 Å)
Cite:Analyses of Variant Acid beta-Glucosidases: EFFECTS OF GAUCHER DISEASE MUTATIONS.
J.Biol.Chem., 281, 2006
2J25
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PARTIALLY DEGLYCOSYLATED GLUCOCERAMIDASE
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, SULFATE ION, ...
Authors:Brumshtein, B., Wormald, M.R., Silman, I., Futerman, A.H., Sussman, J.L.
Deposit date:2006-08-16
Release date:2006-12-06
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.9 Å)
Cite:Structural Comparison of Differently Glycosylated Forms of Acid-Beta-Glucosidase, the Defective Enzyme in Gaucher Disease
Acta Crystallogr.,Sect.D, 62, 2006
2NSX
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STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH PHARMACOLOGICAL CHAPERONE PROVIDES INSIGHT INTO GAUCHER DISEASE
Descriptor:Glucosylceramidase, 2-(ACETYLAMINO)-2-DEOXY-A-D-GLUCOPYRANOSE, SULFATE ION, ...
Authors:Lieberman, R.L., Petsko, G.A., Ringe, D.
Deposit date:2006-11-06
Release date:2006-12-26
Last modified:2017-10-18
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
2NT0
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ACID-BETA-GLUCOSIDASE LOW PH, GLYCEROL BOUND
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, SULFATE ION, ...
Authors:Lieberman, R.L., Petsko, G.A., Ringe, D.
Deposit date:2006-11-06
Release date:2006-12-26
Last modified:2017-10-18
Method:X-RAY DIFFRACTION (1.79 Å)
Cite:Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease.
Nat.Chem.Biol., 3, 2007
2NT1
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STRUCTURE OF ACID-BETA-GLUCOSIDASE AT NEUTRAL PH
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, PHOSPHATE ION
Authors:Lieberman, R.L., Petsko, G.A., Ringe, D.
Deposit date:2006-11-06
Release date:2006-12-26
Last modified:2017-10-18
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
2V3D
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ACID-BETA-GLUCOSIDASE WITH N-BUTYL-DEOXYNOJIRIMYCIN
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, BETA-D-MANNOSE, ...
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:2011-07-13
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
2V3E
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ACID-BETA-GLUCOSIDASE WITH N-NONYL-DEOXYNOJIRIMYCIN
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, BETA-D-MANNOSE, ...
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:2011-07-13
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
2V3F
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ACID-BETA-GLUCOSIDASE PRODUCED IN CARROT
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, ALPHA-D-MANNOSE, ...
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:2015-05-06
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
2WKL
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VELAGLUCERASE ALFA
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, SULFATE ION
Authors:Brumshtein, B., Salinas, P., Peterson, B., Chan, V., Silman, I., Sussman, J.L., Savickas, P.J., Robinson, G.S., Futerman, A.H.
Deposit date:2009-06-15
Release date:2009-09-22
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.7 Å)
Cite:Characterization of Gene-Activated Human Acid-Beta-Glucosidase: Crystal Structure, Glycan Composition and Internalization Into Macrophages.
Glycobiology, 20, 2010
2XWD
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X-RAY STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH 5N,6O-(N'-(N-OCTYL)IMINO)NOJIRIMYCIN IN THE ACTIVE SITE
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, ALPHA-D-MANNOSE, ...
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:2010-11-01
Release date:2011-09-14
Method:X-RAY DIFFRACTION (2.66 Å)
Cite:Cyclodextrin-Mediated Crystallization of Acid Beta-Glucosidase in Complex with Amphiphilic Bicyclic Nojirimycin Analogues.
Org.Biomol.Chem., 9, 2011
2XWE
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X-RAY STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH 5N,6S-(N'-(N-OCTYL)IMINO)-6-THIONOJIRIMYCIN IN THE ACTIVE SITE
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, BETA-D-MANNOSE, ...
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:2017-01-11
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
3GXD
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CRYSTAL STRUCTURE OF APO ACID-BETA-GLUCOSIDASE PH 4.5
Descriptor:Glucosylceramidase, PHOSPHATE ION, N-ACETYL-D-GLUCOSAMINE
Authors:Lieberman, R.L.
Deposit date:2009-04-02
Release date:2009-05-05
Last modified:2017-11-01
Method:X-RAY DIFFRACTION (2.5 Å)
Cite:Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
3GXF
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CRYSTAL STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH ISOFAGOMINE AT NEUTRAL PH
Descriptor:Glucosylceramidase, PHOSPHATE ION, N-ACETYL-D-GLUCOSAMINE, ...
Authors:Lieberman, R.L.
Deposit date:2009-04-02
Release date:2009-05-05
Last modified:2017-11-01
Method:X-RAY DIFFRACTION (2.4 Å)
Cite:Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
3GXI
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CRYSTAL STRUCTURE OF ACID-BETA-GLUCOSIDASE AT PH 5.5
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, PHOSPHATE ION
Authors:Lieberman, R.L.
Deposit date:2009-04-02
Release date:2009-05-05
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (1.84 Å)
Cite:Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
3GXM
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CRYSTAL STRUCTURE OF ACID-BETA-GLUCOSIDASE AT PH 4.5, PHOSPHATE CRYSTALLIZATION CONDITION
Descriptor:Glucosylceramidase, N-ACETYL-D-GLUCOSAMINE, SULFATE ION
Authors:Lieberman, R.L.
Deposit date:2009-04-02
Release date:2009-05-05
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.2 Å)
Cite:Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Biochemistry, 48, 2009
3KE0
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CRYSTAL STRUCTURE OF N370S GLUCOCEREBROSIDASE AT ACIDIC PH.
Descriptor:Glucosylceramidase, 2-(ACETYLAMINO)-2-DEOXY-A-D-GLUCOPYRANOSE, N-ACETYL-D-GLUCOSAMINE, ...
Authors:Wei, R.R., Boucher, S., Pan, C.Q., Edmunds, T.
Deposit date:2009-10-23
Release date:2010-10-27
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.7 Å)
Cite:Crystal Structure of Glucocerebrosidase Containing the N370S Mutation, Implications for Chaperon Therapy
To be Published
3KEH
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CRYSTAL STRUCTURE OF N370S GLUCOCEREBROSIDASE MUTANT AT PH 7.4
Descriptor:Glucocerebrosidase, 2-(ACETYLAMINO)-2-DEOXY-A-D-GLUCOPYRANOSE, SULFATE ION, ...
Authors:Wei, R.R., Boucher, S., Pan, C.Q., Edmunds, T.
Deposit date:2009-10-26
Release date:2010-10-27
Last modified:2011-07-13
Method:X-RAY DIFFRACTION (2.8 Å)
Cite:Crystal Structure of Glucocerebrosidase Containing the N370S mutation: Implication on Chaperon Therapy
To be Published
3RIK
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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:Glucosylceramidase, SULFATE ION, N-ACETYL-D-GLUCOSAMINE, ...
Authors:Orwig, S.D., Lieberman, R.L.
Deposit date:2011-04-13
Release date:2012-03-14
Last modified:2018-08-15
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
3RIL
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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:Glucosylceramidase, SULFATE ION, N-ACETYL-D-GLUCOSAMINE, ...
Authors:Orwig, S.D., Lieberman, R.L.
Deposit date:2011-04-13
Release date:2012-03-14
Last modified:2018-08-15
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
2VT0
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X-RAY STRUCTURE OF A CONJUGATE WITH CONDURITOL-BETA-EPOXIDE OF ACID-BETA-GLUCOSIDASE OVEREXPRESSED IN CULTURED PLANT CELLS
Descriptor:GLUCOSYLCERAMIDASE, (1R,2R,3S,4S,5S,6S)-CYCLOHEXANE-1,2,3,4,5,6-HEXOL, N-ACETYL-D-GLUCOSAMINE, ...
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:2012-10-10
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
2WCG
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X-RAY STRUCTURE OF ACID-BETA-GLUCOSIDASE WITH N-OCTYL(CYCLIC GUANIDINE)-NOJIRIMYCIN IN THE ACTIVE SITE
Descriptor:GLUCOSYLCERAMIDASE, N-ACETYL-D-GLUCOSAMINE, BETA-D-MANNOSE, ...
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:2017-11-15
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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