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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5J5Z
 | | Crystal structure of the D444V disease-causing mutant of the human dihydrolipoamide dehydrogenase | | 分子名称: | Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, ... | | 著者 | Szabo, E, Mizsei, R, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | | 登録日 | 2016-04-04 | | 公開日 | 2017-11-15 | | 最終更新日 | 2024-01-10 | | 実験手法 | X-RAY DIFFRACTION (1.84 Å) | | 主引用文献 | 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
 | | Crystal structure of the human dihydrolipoamide dehydrogenase | | 分子名称: | 2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, ... | | 著者 | Szabo, E, Mizsei, R, Wilk, P, Zambo, Z, Torocsik, B, Weiss, M.S, Adam-Vizi, V, Ambrus, A. | | 登録日 | 2017-03-21 | | 公開日 | 2018-05-16 | | 最終更新日 | 2024-01-17 | | 実験手法 | X-RAY DIFFRACTION (2.27 Å) | | 主引用文献 | 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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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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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
 | | 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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6HG8
 | | Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase. | | 分子名称: | 2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, Dihydrolipoyl dehydrogenase, mitochondrial, ... | | 著者 | Ambrus, A, Szabo, E, Weichsel, A, Bui, D, Wilk, P, Torocsik, B, Weiss, M.S, Montfort, W.R, Jordan, F, Adam-Vizi, V. | | 登録日 | 2018-08-22 | | 公開日 | 2019-09-04 | | 最終更新日 | 2024-01-17 | | 実験手法 | X-RAY DIFFRACTION (1.76 Å) | | 主引用文献 | Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase.
To Be Published
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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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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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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
 | | 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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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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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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