Article
Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain α-ketoacid dehydrogenase kinase.
Proceedings of the National Academy of Sciences of the United States of America - 11 Jun 2013
Tso Shih-Chia, Qi Xiangbing, Gui Wen-Jun, Chuang Jacinta L, Morlock Lorraine K, Wallace Amy L, Ahmed Kamran, Laxman Sunil, Campeau Philippe M, Lee Brendan H, Hutson Susan M, Tu Benjamin P, Williams Noelle S, Tambar Uttam K, Wynn R Max, Chuang David T
Abstract excerpt
The branched-chain amino acids (BCAAs) leucine, isoleucine, and valine are elevated in maple syrup urine disease, heart failure, obesity, and type 2 diabetes. BCAA homeostasis is controlled by the mitochondrial branched-chain α-ketoacid dehydrogenase complex (BCKDC), which is negatively regulated...
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