6I4Q

Crystal structure of the human dihydrolipoamide dehydrogenase at 1.75 Angstrom resolution

Summary for 6I4Q

Entry DOI	10.2210/pdb6i4q/pdb
Descriptor	Dihydrolipoyl dehydrogenase, mitochondrial, FLAVIN-ADENINE DINUCLEOTIDE, 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL, ... (5 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	108224.55
Authors	Nagy, B.,Szabo, E.,Wilk, P.,Torocsik, B.,Weiss, M.S.,Adam-Vizi, V.,Ambrus, A. (deposition date: 2018-11-10, release date: 2019-11-20, Last modification date: 2024-01-24)
Primary citation	Szabo, E.,Wilk, P.,Nagy, B.,Zambo, Z.,Bui, D.,Weichsel, A.,Arjunan, P.,Torocsik, B.,Hubert, A.,Furey, W.,Montfort, W.R.,Jordan, F.,Weiss, M.S.,Adam-Vizi, V.,Ambrus, A. Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants. Hum.Mol.Genet., 28:3339-3354, 2019 Cited by PubMed: 31334547 DOI: 10.1093/hmg/ddz177 PDB entries with the same primary citation
Experimental method	X-RAY DIFFRACTION (1.75 Å)

Structure validation