Crystallographic Investigation and Selective Inhibition of Mutant Isocitrate Dehydrogenase.
Zheng, B., Yao, Y., Liu, Z., Deng, L., Anglin, J.L., Jiang, H., Prasad, B.V., Song, Y.(2013) ACS Med Chem Lett 4: 542-546
- PubMed: 23795241 
- DOI: https://doi.org/10.1021/ml400036z
- Primary Citation of Related Structures:  
4I3K, 4I3L - PubMed Abstract: 
Mutations in isocitrate dehydrogenase (IDH), a key enzyme in the tricarboxylic acid cycle, have recently been found in ~75% glioma and ~20% acute myeloid leukemia. Different from the wild-type enzyme, mutant IDH1 catalyzes the reduction of α-ketoglutaric acid to D -2-hydroxyglutaric acid. Strong evidence has shown mutant IDH1 represents a novel target for this type of cancer. We found two 1-hydroxypyridin-2-one compounds that are potent inhibitors of R132H and R132C IDH1 mutants with K i values as low as 120 nM. These compounds exhibit >60-fold selectivity against wild-type IDH1 and can inhibit the production of D -2-hydroxyglutaric acid in IDH1 mutated cells, representing novel chemical probes for cancer biology studies. We also report the first inhibitor-bound crystal structures of IDH1(R132H), showing these inhibitors have H-bond, electrostatic and hydrophobic interactions with the mutant enzyme. Comparison with the substrate-bound IDH1 structures revealed the structural basis for the high enzyme selectivity of these compounds.
Organizational Affiliation: 
Department of Pharmacology, Baylor College of Medicine, 1 Baylor Plaza, Houston, Texas 77030, United States.