Mitochondrial matters: Mitochondrial bottlenecks, self-assembling structures, and entrapment in the female germline.
Mitochondrial replacement therapy, a procedure to generate embryos with the nuclear genome of a donor mother and the healthy mitochondria of a recipient egg, has recently emerged as a promising strategy to prevent transmission of devastating mitochondrial DNA diseases and infertility. The procedure may produce an embryo that is free of diseased mitochondria. A recent study addresses important fundamental questions about the mechanisms underlying maternal inheritance and translational questions regarding the transgenerational effectiveness of this promising therapeutic strategy. This review considers recent advances in our understanding of maternal inheritance of mitochondria, implications for fertility and mitochondrial disease, and potential roles for the Balbiani body, an ancient oocyte structure, in mitochondrial selection in oocytes, with emphasis on therapies to remedy mitochondrial disorders.
Department of Cell, Developmental and Regenerative Biology, Icahn School of Medicine Mount Sinai, New York, NY 10029-6574, United States; Department of Developmental and Molecular Biology, Albert Einstein College of Medicine, Yeshiva University, Bronx, NY 10461, United States; Department of Neuroscience, Albert Einstein College of Medicine, Yeshiva University, Bronx, NY 10461, United States. Electronic address: florence.marlow@mssm.edu.
Florence L
FL
Marlow
D000818:Animals; D004272:DNA, Mitochondrial / Q000235:genetics; D005260:Female; D005854:Germ Cells / Q000378:metabolism*; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D000069321:Mitochondrial Replacement Therapy; D009865:Oocytes / Q000166:cytology / Q000378:metabolism
D004272:DNA, Mitochondrial