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TitlePyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the alpha V138M variant of human pyruvate dehydrogenase.
Journal, issue, pagesJ. Biol. Chem., Vol. 293, Page 13204-13213, Year 2018
Publish dateFeb 12, 2018 (structure data deposition date)
AuthorsWhitley, M.J. / Arjunan, P. / Nemeria, N.S. / Korotchkina, L.G. / Park, Y.H. / Patel, M.S. / Jordan, F. / Furey, W.
External linksJ. Biol. Chem. / PubMed:29970614
MethodsX-ray diffraction
Resolution2.69 - 2.7 Å
Structure data

PDB-6cer:
Human pyruvate dehydrogenase complex E1 component V138M mutation
Method: X-RAY DIFFRACTION / Resolution: 2.69 Å

PDB-6cfo:
HUMAN PYRUVATE DEHYDROGENASE E1 COMPONENT COMPLEX WITH COVALENT TDP ADDUCT ACETYL PHOSPHINATE
Method: X-RAY DIFFRACTION / Resolution: 2.7 Å

Chemicals

ChemComp-TPP:
THIAMINE DIPHOSPHATE

ChemComp-MG:
Unknown entry

ChemComp-HOH:
WATER

ChemComp-A5X:
3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-2-{(1S)-1-hydroxy-1-[(R)-hydroxy(oxo)-lambda~5~-phosphanyl]ethyl}-5-(2-{[(S)-hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-4-methyl-1,3-thiazol-3-ium

Source
  • homo sapiens (human)
KeywordsOXIDOREDUCTASE / pyruvate dehydrogenase deficiency / mutation / thiamin diphosphate / metabolism / THIAMIN PYROPHOSPHATE / ACETYL PHOSPHINATE / INHIBITOR / PYRUVATE DEHYDROGENASE

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