7ZYT

Crystal structure of the I318T pathogenic variant of the human dihydrolipoamide dehydrogenase

Summary for 7ZYT

Entry DOI	10.2210/pdb7zyt/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	107301.88
Authors	Nemes-Nikodem, E.,Szabo, E.,Vass, K.R.,Lennartz, F.,Nagy, B.,Torocsik, B.,Weiss, M.S.,Adam-Vizi, V.,Ambrus, A. (deposition date: 2022-05-25, release date: 2023-06-14, 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 Cited by PubMed: 37446004 DOI: 10.3390/ijms241310826 PDB entries with the same primary citation
Experimental method	X-RAY DIFFRACTION (2.892 Å)

Structure validation