3HG3
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![BU of 3hg3 by Molmil](/molmil-images/mine/3hg3) | Human alpha-galactosidase catalytic mechanism 2. Substrate bound | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2009-05-13 | Release date: | 2009-11-24 | Last modified: | 2024-04-03 | Method: | X-RAY DIFFRACTION (1.9 Å) | Cite: | Catalytic mechanism of human alpha-galactosidase. J.Biol.Chem., 285, 2010
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8K7F
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![BU of 8k7f by Molmil](/molmil-images/mine/8k7f) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3S,4R,5R)-2,5-bis(hydroxymethyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2,5-bis(hydroxymethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (1.98 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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6IBK
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![BU of 6ibk by Molmil](/molmil-images/mine/6ibk) | Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclosulfamidate ME763 | Descriptor: | (3~{a}~{R},4~{S},5~{S},6~{S},7~{R},7~{a}~{S})-7-(hydroxymethyl)-2,2-bis(oxidanylidene)-3~{a},4,5,6,7,7~{a}-hexahydro-3~{H}-benzo[d][1,2,3]oxathiazole-4,5,6-triol, 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Rowland, R.J, Wu, L, Davies, G.J. | Deposit date: | 2018-11-30 | Release date: | 2019-10-09 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.99 Å) | Cite: | Alpha-d-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal alpha-galactosidase A in Fabry disease Chem Sci, 2019
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8K7L
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![BU of 8k7l by Molmil](/molmil-images/mine/8k7l) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3R,4S,5R)-2-(aminomethyl)-5-(hydroxymethyl)-1-methylpyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2-(aminomethyl)-5-(hydroxymethyl)-1-methyl-pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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8K7K
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![BU of 8k7k by Molmil](/molmil-images/mine/8k7k) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3S,4R,5R)-2,5-bis(hydroxymethyl)-1-methylpyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{S},4~{R},5~{R})-2,5-bis(hydroxymethyl)-1-methyl-pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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3S5Z
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![BU of 3s5z by Molmil](/molmil-images/mine/3s5z) | Pharmacological Chaperoning in Human alpha-Galactosidase | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, GLYCEROL, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2011-05-23 | Release date: | 2012-01-04 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.001 Å) | Cite: | The molecular basis of pharmacological chaperoning in human alpha-galactosidase Chem.Biol., 18, 2011
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8K7J
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![BU of 8k7j by Molmil](/molmil-images/mine/8k7j) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3R,4S,5R)-2-((dimethylamino)methyl)-5-(hydroxymethyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2-[(dimethylamino)methyl]-5-(hydroxymethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.01 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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6IBR
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![BU of 6ibr by Molmil](/molmil-images/mine/6ibr) | Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclophellitol epoxide LWA481 | Descriptor: | (2~{R},3~{S},4~{S},5~{R},6~{S})-5-(hydroxymethyl)-7-oxabicyclo[4.1.0]heptane-2,3,4-triol, 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Rowland, R.J, Wu, L, Davies, G.J. | Deposit date: | 2018-11-30 | Release date: | 2019-10-09 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (2.02 Å) | Cite: | Alpha-d-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal alpha-galactosidase A in Fabry disease Chem Sci, 2019
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3LX9
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![BU of 3lx9 by Molmil](/molmil-images/mine/3lx9) | Interconversion of Human Lysosomal Enzyme Specificities | Descriptor: | 2-acetamido-2-deoxy-alpha-D-galactopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, ... | Authors: | Tomasic, I.B, Metcalf, M.C, Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2010-02-25 | Release date: | 2010-05-05 | Last modified: | 2023-09-06 | Method: | X-RAY DIFFRACTION (2.04 Å) | Cite: | Interconversion of the specificities of human lysosomal enzymes associated with Fabry and Schindler diseases. J.Biol.Chem., 285, 2010
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6IBT
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![BU of 6ibt by Molmil](/molmil-images/mine/6ibt) | Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclophellitol aziridine ME737 | Descriptor: | (1~{S},2~{S},3~{S},4~{S},5~{R},6~{S})-5-(hydroxymethyl)-7-azabicyclo[4.1.0]heptane-2,3,4-triol, 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Rowland, R.J, Wu, L, Davies, G.J. | Deposit date: | 2018-11-30 | Release date: | 2019-10-09 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (2.04 Å) | Cite: | Alpha-d-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal alpha-galactosidase A in Fabry disease Chem Sci, 2019
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6IBM
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![BU of 6ibm by Molmil](/molmil-images/mine/6ibm) | Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclosulfate ME776 | Descriptor: | 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ACETATE ION, ... | Authors: | Rowland, R.J, Wu, L, Davies, G.J. | Deposit date: | 2018-11-30 | Release date: | 2019-10-09 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (2.07 Å) | Cite: | Alpha-d-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal alpha-galactosidase A in Fabry disease Chem Sci, 2019
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3S5Y
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![BU of 3s5y by Molmil](/molmil-images/mine/3s5y) | Pharmacological Chaperoning in Human alpha-Galactosidase | Descriptor: | (2R,3S,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol, 1,2-ETHANEDIOL, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2011-05-23 | Release date: | 2012-01-04 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.105 Å) | Cite: | The molecular basis of pharmacological chaperoning in human alpha-galactosidase Chem.Biol., 18, 2011
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8K7I
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![BU of 8k7i by Molmil](/molmil-images/mine/8k7i) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3S,4R,5R)-2-(hydroxymethyl)-5-((methylamino)methyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{S},4~{R},5~{R})-2-(hydroxymethyl)-5-(methylaminomethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.12 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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8K7E
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![BU of 8k7e by Molmil](/molmil-images/mine/8k7e) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3R,4S,5R)-2-(aminomethyl)-5-(hydroxymethyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2-(aminomethyl)-5-(hydroxymethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.2 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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3GXP
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![BU of 3gxp by Molmil](/molmil-images/mine/3gxp) | Crystal structure of acid-alpha-galactosidase A complexed with galactose at pH 4.5 | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, ... | Authors: | Lieberman, R.L. | Deposit date: | 2009-04-02 | Release date: | 2009-05-05 | Last modified: | 2020-07-29 | Method: | X-RAY DIFFRACTION (2.2 Å) | Cite: | Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability. Biochemistry, 48, 2009
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8K7H
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![BU of 8k7h by Molmil](/molmil-images/mine/8k7h) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3S,4R)-2-(hydroxymethyl)-1-methylpyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{S},4~{R})-2-(hydroxymethyl)-1-methyl-pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.28 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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3HG2
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![BU of 3hg2 by Molmil](/molmil-images/mine/3hg2) | Human alpha-galactosidase catalytic mechanism 1. Empty active site | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, ACETIC ACID, Alpha-galactosidase A, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2009-05-13 | Release date: | 2009-11-24 | Last modified: | 2023-09-06 | Method: | X-RAY DIFFRACTION (2.3 Å) | Cite: | Catalytic mechanism of human alpha-galactosidase. J.Biol.Chem., 285, 2010
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3HG4
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![BU of 3hg4 by Molmil](/molmil-images/mine/3hg4) | Human alpha-galactosidase catalytic mechanism 3. Covalent intermediate | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-6)]2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2009-05-13 | Release date: | 2009-11-24 | Last modified: | 2024-04-03 | Method: | X-RAY DIFFRACTION (2.3 Å) | Cite: | Catalytic mechanism of human alpha-galactosidase. J.Biol.Chem., 285, 2010
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3HG5
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![BU of 3hg5 by Molmil](/molmil-images/mine/3hg5) | Human alpha-galactosidase catalytic mechanism 4. Product bound | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, ACETIC ACID, Alpha-galactosidase A, ... | Authors: | Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2009-05-13 | Release date: | 2009-11-24 | Last modified: | 2024-04-03 | Method: | X-RAY DIFFRACTION (2.3 Å) | Cite: | Catalytic mechanism of human alpha-galactosidase. J.Biol.Chem., 285, 2010
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8K7G
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![BU of 8k7g by Molmil](/molmil-images/mine/8k7g) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3S,4R,5R)-2,5-bis(hydroxymethyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2,5-bis(hydroxymethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.32 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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3LXC
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![BU of 3lxc by Molmil](/molmil-images/mine/3lxc) | Interconversion of Human Lysosomal Enzyme Specificities | Descriptor: | 2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, GLYCEROL, ... | Authors: | Tomasic, I.B, Metcalf, M.C, Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2010-02-25 | Release date: | 2010-05-05 | Last modified: | 2023-09-06 | Method: | X-RAY DIFFRACTION (2.35 Å) | Cite: | Interconversion of the specificities of human lysosomal enzymes associated with Fabry and Schindler diseases. J.Biol.Chem., 285, 2010
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4NXS
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![BU of 4nxs by Molmil](/molmil-images/mine/4nxs) | Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT | Descriptor: | (2R,3S,4R,5S)-N-(4-fluorophenyl)-3,4,5-trihydroxy-2-(hydroxymethyl)piperidine-1-carbothioamide, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Johnson, J.L, Drury, J.E, Lieberman, R.L. | Deposit date: | 2013-12-09 | Release date: | 2014-06-11 | Last modified: | 2023-09-20 | Method: | X-RAY DIFFRACTION (2.5493 Å) | Cite: | Molecular Basis of 1-Deoxygalactonojirimycin Arylthiourea Binding to Human alpha-Galactosidase A: Pharmacological Chaperoning Efficacy on Fabry Disease Mutants. Acs Chem.Biol., 9, 2014
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8K7D
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![BU of 8k7d by Molmil](/molmil-images/mine/8k7d) | Crystal structure of human lysosomal alpha-galactosidase A in complex with (2R,3R,4S,5R)-2-(aminomethyl)-5-(hydroxymethyl)pyrrolidine-3,4-diol | Descriptor: | (2~{R},3~{R},4~{S},5~{R})-2-(aminomethyl)-5-(hydroxymethyl)pyrrolidine-3,4-diol, 2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... | Authors: | Li, H.Y, Huang, K.F, Ko, T.P, Cheng, W.C. | Deposit date: | 2023-07-26 | Release date: | 2024-04-10 | Method: | X-RAY DIFFRACTION (2.61 Å) | Cite: | Mechanistic Insights into Dibasic Iminosugars as pH-Selective Pharmacological Chaperones to Stabilize Human alpha-Galactosidase. Jacs Au, 4, 2024
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3TV8
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![BU of 3tv8 by Molmil](/molmil-images/mine/3tv8) | Pharmacological Chaperoning in Human alpha-Galactosidase | Descriptor: | (2R,3S,4R,5S)-2-(hydroxymethyl)piperidine-3,4,5-triol, 2-acetamido-2-deoxy-beta-D-glucopyranose, Alpha-galactosidase A, ... | Authors: | Rogich, J.J, Guce, A.I, Clark, N.E, Garman, S.C. | Deposit date: | 2011-09-19 | Release date: | 2012-01-04 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.639 Å) | Cite: | The molecular basis of pharmacological chaperoning in human alpha-galactosidase Chem.Biol., 18, 2011
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3GXT
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![BU of 3gxt by Molmil](/molmil-images/mine/3gxt) | |