7PSC

Crystal structure of the disease-causing I358T mutant of the human dihydrolipoamide dehydrogenase

Summary for 7PSC

• Entry DOI: 10.2210/pdb7psc/pdb
• Descriptor: Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, ... (4 entities in total)
• Functional Keywords: lipoamide dehydrogenase, pathogenic mutation, e3 deficiency, alpha-ketoglutarate dehydrogenase complex, 2-oxoglutarate dehydrogenase complex, pyruvate dehydrogenase complex, oxidoreductase
• Biological source: Homo sapiens (Human)
• Total number of polymer chains: 2
• Total formula weight: 107658.29

Authors

Nemes-Nikodem, E.,Szabo, E.,Zambo, Z.,Vass, K.R.,Taberman, H.,Torocsik, B.,Weiss, M.S.,Adam-Vizi, V.,Ambrus, A. (deposition date: 2021-09-22, release date: 2023-04-05, Last modification date: 2024-02-07)

• Primary citation: Szabo, E.,Nemes-Nikodem, E.,Vass, K.R.,Zambo, Z.,Zrupko, E.,Torocsik, B.,Ozohanics, O.,Nagy, B.,Ambrus, A.; Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase.; Int J Mol Sci, 24:-, 2023; PubMed: 37446004; DOI: 10.3390/ijms241310826

Experimental method

X-RAY DIFFRACTION (2.436 Å)