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] | 分子名称: | Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial | 著者 | Nagy, B, Zambo, Z, Hubert, A, Polak, M, Nemeria, N.S, Novacek, J, Jordan, F, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-07-06 | 公開日 | 2020-01-22 | 最終更新日 | 2024-05-15 | 実験手法 | ELECTRON MICROSCOPY (2.9 Å) | 主引用文献 | 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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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 | 分子名称: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | 著者 | Nagy, B, Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.75 Å) | 主引用文献 | 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 | 分子名称: | 1,2-ETHANEDIOL, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, ... | 著者 | Nemes-Nikodem, E, Szabo, E, Vass, K.R, Lennartz, F, Nagy, B, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2022-05-25 | 公開日 | 2023-06-14 | 最終更新日 | 2024-02-07 | 実験手法 | X-RAY DIFFRACTION (2.892 Å) | 主引用文献 | Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase. Int J Mol Sci, 24, 2023
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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 | 分子名称: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | 著者 | Szabo, E, Wilk, P, Bui, D, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.439 Å) | 主引用文献 | 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 | 分子名称: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | 著者 | Szabo, E, Wilk, P, Hubert, A, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.75 Å) | 主引用文献 | 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 | 分子名称: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | 著者 | Szabo, E, Wilk, P, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.823 Å) | 主引用文献 | 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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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 | 分子名称: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | 著者 | Nemes-Nikodem, E, Szabo, E, Zambo, Z, Vass, K.R, Taberman, H, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2021-09-22 | 公開日 | 2023-04-05 | 最終更新日 | 2024-02-07 | 実験手法 | X-RAY DIFFRACTION (2.436 Å) | 主引用文献 | Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase. Int J Mol Sci, 24, 2023
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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 | 分子名称: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | 著者 | Szabo, E, Wilk, P, Hubert, A, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.84 Å) | 主引用文献 | 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 | 分子名称: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | 著者 | Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-12 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (2.342 Å) | 主引用文献 | 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 | 分子名称: | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | 著者 | Szabo, E, Wilk, P, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | 登録日 | 2018-11-10 | 公開日 | 2019-11-20 | 最終更新日 | 2024-01-24 | 実験手法 | X-RAY DIFFRACTION (1.6 Å) | 主引用文献 | 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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