The mitochondrial permeability transition pore (mPTP) is a source of proton leak and is physiologically open in the developing wild-type heart. Newborn Fragile X syndrome (FXS) cardiomyocyte mitochondria have excess coenzyme Q (CoQ), less proton leak, and a closed mPTP. CoQ is likely an important regulator of the mPTP duing development.
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Barajas, M. et al. The newborn Fmr1 knockout mouse: a novel model of excess ubiquinone and closed mitochondrial permeability transition pore in the developing heart. Pediatr. Res. https://doi.org/10.1038/s41390-020-1064-6 (2020).
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This work was supported by NIH/NINDS R01NS112706 (to R.J.L.).
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Barajas, M., Wang, A., Griffiths, K.K. et al. Insights image for “The newborn Fmr1 knockout mouse: a novel model of excess ubiquinone and closed mitochondrial permeability transition pore in the developing heart”. Pediatr Res 89, 707 (2021). https://doi.org/10.1038/s41390-020-01144-4
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DOI: https://doi.org/10.1038/s41390-020-01144-4
