Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations.

doi:10.1016/j.cmet.2018.03.002

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Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations.

Cell metabolism - 1 May 2018

Chen Qiuying, Kirk Kathryne, Shurubor Yevgeniya I, Zhao Dazhi, Arreguin Andrea J, Shahi Ifrah, Valsecchi Federica, Primiano Guido, Calder Elizabeth L, Carelli Valerio, Denton Travis T, Beal M Flint, Gross Steven S, Manfredi Giovanni, D'Aurelio Marilena

Abstract excerpt

Using molecular, biochemical, and untargeted stable isotope tracing approaches, we identify a previously unappreciated glutamine-derived α-ketoglutarate (αKG) energy-generating anaplerotic flux to be critical in mitochondrial DNA (mtDNA) mutant cells that harbor human disease-associated oxidative phosphorylation defects. Stimulating this flux with αKG supplementation enables the survival of diverse mtDNA mutant...

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Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations.

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