6CER
Human pyruvate dehydrogenase complex E1 component V138M mutation
Summary for 6CER
Entry DOI | 10.2210/pdb6cer/pdb |
Related | 1NI4 |
Descriptor | Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial, Pyruvate dehydrogenase E1 component subunit beta, mitochondrial, THIAMINE DIPHOSPHATE, ... (5 entities in total) |
Functional Keywords | pyruvate dehydrogenase deficiency, mutation, thiamin diphosphate, metabolism, oxidoreductase |
Biological source | Homo sapiens (Human) More |
Total number of polymer chains | 8 |
Total formula weight | 309077.83 |
Authors | Whitley, M.J.,Arjunan, P.,Furey, W. (deposition date: 2018-02-12, release date: 2018-07-11, Last modification date: 2023-10-04) |
Primary citation | Whitley, M.J.,Arjunan, P.,Nemeria, N.S.,Korotchkina, L.G.,Park, Y.H.,Patel, M.S.,Jordan, F.,Furey, W. Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the alpha V138M variant of human pyruvate dehydrogenase. J. Biol. Chem., 293:13204-13213, 2018 Cited by PubMed: 29970614DOI: 10.1074/jbc.RA118.003996 PDB entries with the same primary citation |
Experimental method | X-RAY DIFFRACTION (2.69 Å) |
Structure validation
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