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Structure paper

TitleStructural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase.
Journal, issue, pagesInt J Mol Sci, Vol. 24, Year 2023
Publish dateSep 22, 2021 (structure data deposition date)
AuthorsSzabo, E. / Nemes-Nikodem, E. / Vass, K.R. / Zambo, Z. / Zrupko, E. / Torocsik, B. / Ozohanics, O. / Nagy, B. / Ambrus, A.
External linksInt J Mol Sci / PubMed:37446004
MethodsX-ray diffraction
Resolution2.436 - 2.892 Å
Structure data

PDB-7psc:
Crystal structure of the disease-causing I358T mutant of the human dihydrolipoamide dehydrogenase
Method: X-RAY DIFFRACTION / Resolution: 2.436 Å

PDB-7zyt:
Crystal structure of the I318T pathogenic variant of the human dihydrolipoamide dehydrogenase
Method: X-RAY DIFFRACTION / Resolution: 2.892 Å

Chemicals

ChemComp-FAD:
FLAVIN-ADENINE DINUCLEOTIDE / FAD*YM

ChemComp-BTB:
2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL / pH buffer*YM

ChemComp-HOH:
WATER

ChemComp-EDO:
1,2-ETHANEDIOL

Source
  • homo sapiens (human)
KeywordsOXIDOREDUCTASE / lipoamide dehydrogenase / pathogenic mutation / E3 deficiency / alpha-ketoglutarate dehydrogenase complex / 2-oxoglutarate dehydrogenase complex / pyruvate dehydrogenase complex

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