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, 2013). Families with known mtDNA mutations who are searching for reproductive options will likely be better served by emerging methodologies that do not just select, but actively eliminate, mutant mtDNA in oocytes. In particular, spindle transfer (Lee et?al., 2012?and?Tachibana Rucaparib in vitro et?al., 2013) results in Fluconazole options for the prevention of intergenerational transmission of mtDNA diseases (Lee et?al., 2012?and?Tachibana et?al., 2013). We also highlight a need for the development of additional animal models of naturally arising pathogenic mtDNA mutations that will be essential for a better understanding of human biology and disease. We hope that the work of Steffann et?al. and our response will contribute to a clearer understanding of the complexities in predicting mtDNA disease transmission and serve as a useful platform to stimulate further discussions. This work was supported by National Institutes of?Health grants R01-HD063276, R01-HD057121, R01-HD059946, R01-EY021214, P51-OD011092 (S.M.), and R03-DK082446 (M.J.F.), as well as a grant from the Leducq Foundation (S.M.). The content is solely the responsibility of the authors and does not necessarily represent the official views of Selleck ALK inhibitor the National Institutes of Health. ""Steroids are hormones that are involved in?diverse physiological functions ranging from regulation of metabolism, behavior, and fertility to inflammation and immune control. The different forms of steroids (progesterone, mineralocorticoids, glucocorticoids [GCs], androgens, and estrogens) all (1) form by a synthesis pathway that starts with conversion of cholesterol into pregnenolone, (2) are to?some degree anti-inflammatory and immunosuppressive, (3) interact with a specific nuclear receptor, and (4) can act on distant tissues.