A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans.
A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans.
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DOI：
10.1126/science.1218099
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发表时间：
2012-07-06
期刊：
影响因子：
--
通讯作者：
Rutter J
中科院分区：
文献类型：
--
作者：
Bricker DK;Taylor EB;Schell JC;Orsak T;Boutron A;Chen YC;Cox JE;Cardon CM;Van Vranken JG;Dephoure N;Redin C;Boudina S;Gygi SP;Brivet M;Thummel CS;Rutter J
Pyruvate constitutes a critical branch point in cellular carbon metabolism. We have identified two proteins, Mpc1 and Mpc2, as essential for mitochondrial pyruvate transport in yeast, Drosophila, and humans. Mpc1 and Mpc2 associate to form an ~150-kilodalton complex in the inner mitochondrial membrane. Yeast and Drosophila mutants lacking MPC1 display impaired pyruvate metabolism, with an accumulation of upstream metabolites and a depletion of tricarboxylic acid cycle intermediates. Loss of yeast Mpc1 results in defective mitochondrial pyruvate uptake, and silencing of MPC1 or MPC2 in mammalian cells impairs pyruvate oxidation. A point mutation in MPC1 provides resistance to a known inhibitor of the mitochondrial pyruvate carrier. Human genetic studies of three families with children suffering from lactic acidosis and hyperpyruvatemia revealed a causal locus that mapped to MPC1, changing single amino acids that are conserved throughout eukaryotes. These data demonstrate that Mpc1 and Mpc2 form an essential part of the mitochondrial pyruvate carrier.
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