Mitochondrial genome inheritance and replacement in the human germline.

Publisher: Wiley Blackwell Country of Publication: England NLM ID: 8208664 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1460-2075 (Electronic) Linking ISSN: 02614189 NLM ISO Abbreviation: EMBO J Subsets: MEDLINE

Publication: 2014- : London : Wiley Blackwell
Original Publication: Eynsham, Oxford, England : Published for the European Molecular Biology Organization by IRL Press, [c1982-

DNA Replication* ; Genome, Human* ; Genome, Mitochondrial* ; Germ Cells* ; Wills*; Humans ; Mitochondrial Replacement Therapy/methods ; Models, Biological

Mitochondria, the ubiquitous power packs in nearly every eukaryotic cell, contain their own DNA, known as mtDNA, which is inherited exclusively from the mother. The number of mitochondrial genomes varies depending on the cell's energy needs. The mature oocyte contains the highest number of mitochondria of any cell type, although there is little if any mtDNA replication after fertilization until the embryo implants. This has potential repercussions for mitochondrial replacement therapy (MRT; see description of currently employed methods below) used to prevent the transmission of mtDNA-based disorders. If only a few mitochondria with defective mtDNA are left in the embryo and undergo extensive replication, it might therefore thwart the purpose of MRT In order to improve the safety and efficacy of this experimental therapy, we need a better understanding of how and which mtDNA is tagged for replication versus transcription after fertilization of the oocyte.
(© 2017 The Authors.)

Erratum in: EMBO J. 2017 Sep 1;36(17 ):2659. (PMID: 28864679)

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P51 OD011092 United States OD NIH HHS

Date Created: 20170707 Date Completed: 20170811 Latest Revision: 20181113

20240829

PMC5538763

10.15252/embj.201797606

28679504