6I4R
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![BU of 6i4r by Molmil](/molmil-images/mine/6i4r) | Crystal structure of the disease-causing R460G mutant of the human dihydrolipoamide dehydrogenase at 1.44 Angstrom resolution | Descriptor: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | Authors: | Szabo, E, Wilk, P, Bui, D, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.439 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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6I4S
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![BU of 6i4s by Molmil](/molmil-images/mine/6i4s) | Crystal structure of the disease-causing R447G mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | Authors: | Szabo, E, Wilk, P, Hubert, A, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.75 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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6I4T
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![BU of 6i4t by Molmil](/molmil-images/mine/6i4t) | Crystal structure of the disease-causing I445M mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | Authors: | Szabo, E, Wilk, P, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.823 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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1AMH
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![BU of 1amh by Molmil](/molmil-images/mine/1amh) | UNCOMPLEXED RAT TRYPSIN MUTANT WITH ASP 189 REPLACED WITH SER (D189S) | Descriptor: | ANIONIC TRYPSIN, CALCIUM ION | Authors: | Szabo, E, Bocskei, Z.S, Naray-Szabo, G, Graf, L. | Deposit date: | 1997-06-17 | Release date: | 1997-12-24 | Last modified: | 2023-08-02 | Method: | X-RAY DIFFRACTION (2.5 Å) | Cite: | The three-dimensional structure of Asp189Ser trypsin provides evidence for an inherent structural plasticity of the protease. Eur.J.Biochem., 263, 1999
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1KDQ
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![BU of 1kdq by Molmil](/molmil-images/mine/1kdq) | Crystal Structure Analysis of the Mutant S189D Rat Chymotrypsin | Descriptor: | CALCIUM ION, CHYMOTRYPSIN B, B CHAIN, ... | Authors: | Szabo, E, Bocskei, Z, Naray-Szabo, G, Graf, L, Venekei, I. | Deposit date: | 2001-11-13 | Release date: | 2003-06-10 | Last modified: | 2021-10-27 | Method: | X-RAY DIFFRACTION (2.55 Å) | Cite: | Three Dimensional Structures of S189D Chymotrypsin and D189S Trypsin Mutants: The Effect of Polarity at Site 189 on a Protease-specific Stabilization of
the Substrate-binding Site J.Mol.Biol., 331, 2003
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5J5Z
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![BU of 5j5z by Molmil](/molmil-images/mine/5j5z) | Crystal structure of the D444V disease-causing mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | Authors: | Szabo, E, Mizsei, R, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2016-04-04 | Release date: | 2017-11-15 | Last modified: | 2024-01-10 | Method: | X-RAY DIFFRACTION (1.84 Å) | Cite: | Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase. Free Radic. Biol. Med., 124, 2018
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5NHG
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![BU of 5nhg by Molmil](/molmil-images/mine/5nhg) | Crystal structure of the human dihydrolipoamide dehydrogenase | Descriptor: | 2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, ... | Authors: | Szabo, E, Mizsei, R, Wilk, P, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2017-03-21 | Release date: | 2018-05-16 | Last modified: | 2024-01-17 | Method: | X-RAY DIFFRACTION (2.27 Å) | Cite: | Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase. Free Radic. Biol. Med., 124, 2018
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6I4U
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![BU of 6i4u by Molmil](/molmil-images/mine/6i4u) | Crystal structure of the disease-causing G426E mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | Authors: | Szabo, E, Wilk, P, Hubert, A, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.84 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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6I4Z
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![BU of 6i4z by Molmil](/molmil-images/mine/6i4z) | Crystal structure of the disease-causing P453L mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | Authors: | Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-12 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (2.342 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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6I4P
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![BU of 6i4p by Molmil](/molmil-images/mine/6i4p) | Crystal structure of the disease-causing G194C mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | Authors: | Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.6 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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7ZYT
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![BU of 7zyt by Molmil](/molmil-images/mine/7zyt) | Crystal structure of the I318T pathogenic variant of the human dihydrolipoamide dehydrogenase | Descriptor: | 1,2-ETHANEDIOL, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, ... | Authors: | Nemes-Nikodem, E, Szabo, E, Vass, K.R, Lennartz, F, Nagy, B, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2022-05-25 | Release date: | 2023-06-14 | Last modified: | 2024-02-07 | Method: | X-RAY DIFFRACTION (2.892 Å) | Cite: | Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase. Int J Mol Sci, 24, 2023
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7PSC
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![BU of 7psc by Molmil](/molmil-images/mine/7psc) | Crystal structure of the disease-causing I358T mutant of the human dihydrolipoamide dehydrogenase | Descriptor: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | Authors: | Nemes-Nikodem, E, Szabo, E, Zambo, Z, Vass, K.R, Taberman, H, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2021-09-22 | Release date: | 2023-04-05 | Last modified: | 2024-02-07 | Method: | X-RAY DIFFRACTION (2.436 Å) | Cite: | Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase. Int J Mol Sci, 24, 2023
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6I4Q
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![BU of 6i4q by Molmil](/molmil-images/mine/6i4q) | Crystal structure of the human dihydrolipoamide dehydrogenase at 1.75 Angstrom resolution | Descriptor: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | Authors: | Nagy, B, Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-11-10 | Release date: | 2019-11-20 | Last modified: | 2024-01-24 | Method: | X-RAY DIFFRACTION (1.75 Å) | Cite: | Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28, 2019
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1XLM
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![BU of 1xlm by Molmil](/molmil-images/mine/1xlm) | D254E, D256E MUTANT OF D-XYLOSE ISOMERASE COMPLEXED WITH AL3 AND XYLITOL | Descriptor: | ALUMINUM ION, D-XYLOSE ISOMERASE, Xylitol | Authors: | Gerczei, T, Bocskei, Z.S, Szabo, E, Naray-Szabo, G, Asboth, B. | Deposit date: | 1997-07-22 | Release date: | 1998-01-28 | Last modified: | 2024-05-22 | Method: | X-RAY DIFFRACTION (2.4 Å) | Cite: | Structure determination and refinement of the Al3+ complex of the D254,256E mutant of Arthrobacter D-xylose isomerase at 2.40 A resolution. Further evidence for inhibitor-induced metal ion movement. Int.J.Biol.Macromol., 25, 1999
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6HG8
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![BU of 6hg8 by Molmil](/molmil-images/mine/6hg8) | Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase. | Descriptor: | 2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | Authors: | Ambrus, A, Szabo, E, Weichsel, A, Bui, D, Wilk, P, Torocsik, B, Weiss, M.S, Montfort, W.R, Jordan, F, Adam-Vizi, V. | Deposit date: | 2018-08-22 | Release date: | 2019-09-04 | Last modified: | 2024-01-17 | Method: | X-RAY DIFFRACTION (1.76 Å) | Cite: | Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase. To Be Published
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6H05
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![BU of 6h05 by Molmil](/molmil-images/mine/6h05) | Cryo-electron microscopic structure of the dihydrolipoamide succinyltransferase (E2) component of the human alpha-ketoglutarate (2-oxoglutarate) dehydrogenase complex [residues 218-453] | Descriptor: | Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial | Authors: | Nagy, B, Zambo, Z, Hubert, A, Polak, M, Nemeria, N.S, Novacek, J, Jordan, F, Adam-Vizi, V, Ambrus, A. | Deposit date: | 2018-07-06 | Release date: | 2020-01-22 | Last modified: | 2024-05-15 | Method: | ELECTRON MICROSCOPY (2.9 Å) | Cite: | Structure of the dihydrolipoamide succinyltransferase (E2) component of the human alpha-ketoglutarate dehydrogenase complex (hKGDHc) revealed by cryo-EM and cross-linking mass spectrometry: Implications for the overall hKGDHc structure. Biochim Biophys Acta Gen Subj, 1865, 2021
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3RFN
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![BU of 3rfn by Molmil](/molmil-images/mine/3rfn) | Epitope backbone grafting by computational design for improved presentation of linear epitopes on scaffold proteins | Descriptor: | BB_1wnu_001, ZINC ION | Authors: | Azoitei, M.L, Ban, Y.A, Julien, J.P, Bryson, S, Schroeter, A, Kalyuzhniy, O, Porter, J.R, Adachi, Y, Baker, D, Szabo, E, Pai, E.F, Schief, W.R. | Deposit date: | 2011-04-06 | Release date: | 2011-11-09 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (1.8 Å) | Cite: | Computational design of high-affinity epitope scaffolds by backbone grafting of a linear epitope. J.Mol.Biol., 415, 2012
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3RHU
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![BU of 3rhu by Molmil](/molmil-images/mine/3rhu) | Epitope backbone grafting by computational design for improved presentation of linear epitopes on scaffold proteins | Descriptor: | SC_1wnu | Authors: | Azoitei, M.L, Ban, Y.A, Julien, J.P, Bryson, S, Schroeter, A, Kalyuzhniy, O, Porter, J.R, Adachi, Y, Baker, D, Szabo, E, Pai, E.F, Schief, W.R. | Deposit date: | 2011-04-12 | Release date: | 2011-11-09 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.8 Å) | Cite: | Computational design of high-affinity epitope scaffolds by backbone grafting of a linear epitope. J.Mol.Biol., 415, 2012
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3RI0
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![BU of 3ri0 by Molmil](/molmil-images/mine/3ri0) | Epitope backbone grafting by computational design for improved presentation of linear epitopes on scaffold proteins | Descriptor: | BB_2cx5_001, GLYCEROL, SULFATE ION | Authors: | Azoitei, M.L, Ban, Y.A, Julien, J.P, Bryson, S, Schroeter, A, Kalyuzhniy, O, Porter, J.R, Adachi, Y, Baker, D, Szabo, E, Pai, E.F, Schief, W.R. | Deposit date: | 2011-04-12 | Release date: | 2011-11-09 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.25 Å) | Cite: | Computational design of high-affinity epitope scaffolds by backbone grafting of a linear epitope. J.Mol.Biol., 415, 2012
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3RIJ
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![BU of 3rij by Molmil](/molmil-images/mine/3rij) | Epitope backbone grafting by computational design for improved presentation of linear epitopes on scaffold proteins | Descriptor: | GLYCEROL, SC_2cx5 | Authors: | Azoitei, M.L, Ban, Y.A, Julien, J.P, Bryson, S, Schroeter, A, Kalyuzhniy, O, Porter, J.R, Adachi, Y, Baker, D, Szabo, E, Pai, E.F, Schief, W.R. | Deposit date: | 2011-04-13 | Release date: | 2011-11-09 | Last modified: | 2023-09-13 | Method: | X-RAY DIFFRACTION (2.3 Å) | Cite: | Computational design of high-affinity epitope scaffolds by backbone grafting of a linear epitope. J.Mol.Biol., 415, 2012
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