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Embryonic Lethality of Mitochondrial Pyruvate Carrier 1 Deficient Mouse Can Be Rescued by a Ketogenic Diet


The tight control of cellular metabolism and energy production plays a crucial role during embryonic development, cancer and neurodegenerative disorders. We show that mitochondrial pyruvate carrier deficiency in mice causes metabolic alterations that result in lactic acidosis, neurotransmitter imbalance, energy deficit, brain damage and embryonic lethality. Feeding the pregnant dams a ketogenic diet allowed the survival of affected embryos until birth. Our results demonstrate the importance of the mitochondrial pyruvate carrier in maintaining the metabolic program necessary to sustain normal mammalian development.


Vyšlo v časopise: Embryonic Lethality of Mitochondrial Pyruvate Carrier 1 Deficient Mouse Can Be Rescued by a Ketogenic Diet. PLoS Genet 12(5): e32767. doi:10.1371/journal.pgen.1006056
Kategorie: Research Article
prolekare.web.journal.doi_sk: https://doi.org/10.1371/journal.pgen.1006056

Souhrn

The tight control of cellular metabolism and energy production plays a crucial role during embryonic development, cancer and neurodegenerative disorders. We show that mitochondrial pyruvate carrier deficiency in mice causes metabolic alterations that result in lactic acidosis, neurotransmitter imbalance, energy deficit, brain damage and embryonic lethality. Feeding the pregnant dams a ketogenic diet allowed the survival of affected embryos until birth. Our results demonstrate the importance of the mitochondrial pyruvate carrier in maintaining the metabolic program necessary to sustain normal mammalian development.


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