1DTW
HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE
Summary for 1DTW
Entry DOI | 10.2210/pdb1dtw/pdb |
Descriptor | BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE ALPHA SUBUNIT, BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE BETA SUBUNIT, MAGNESIUM ION, ... (6 entities in total) |
Functional Keywords | thdp-binding fold, branched-chain alpha-keto acid dehydrogenase, oxidoreductase |
Biological source | Homo sapiens (human) More |
Cellular location | Mitochondrion matrix: P12694 P21953 |
Total number of polymer chains | 2 |
Total formula weight | 84001.18 |
Authors | AEvarsson, A.,Chuang, J.L.,Wynn, R.M.,Turley, S.,Chuang, D.T.,Hol, W.G.J. (deposition date: 2000-01-13, release date: 2000-03-27, Last modification date: 2024-02-07) |
Primary citation | AEvarsson, A.,Chuang, J.L.,Wynn, R.M.,Turley, S.,Chuang, D.T.,Hol, W.G. Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. Structure Fold.Des., 8:277-291, 2000 Cited by PubMed: 10745006DOI: 10.1016/S0969-2126(00)00105-2 PDB entries with the same primary citation |
Experimental method | X-RAY DIFFRACTION (2.7 Å) |
Structure validation
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