Regulation of Mother-to-Offspring Transmission of mtDNA Heteroplasmy.


Journal

Cell metabolism
ISSN: 1932-7420
Titre abrégé: Cell Metab
Pays: United States
ID NLM: 101233170

Informations de publication

Date de publication:
03 12 2019
Historique:
received: 22 01 2019
revised: 12 06 2019
accepted: 10 09 2019
pubmed: 8 10 2019
medline: 22 9 2020
entrez: 8 10 2019
Statut: ppublish

Résumé

mtDNA is present in multiple copies in each cell derived from the expansions of those in the oocyte. Heteroplasmy, more than one mtDNA variant, may be generated by mutagenesis, paternal mtDNA leakage, and novel medical technologies aiming to prevent inheritance of mtDNA-linked diseases. Heteroplasmy phenotypic impact remains poorly understood. Mouse studies led to contradictory models of random drift or haplotype selection for mother-to-offspring transmission of mtDNA heteroplasmy. Here, we show that mtDNA heteroplasmy affects embryo metabolism, cell fitness, and induced pluripotent stem cell (iPSC) generation. Thus, genetic and pharmacological interventions affecting oxidative phosphorylation (OXPHOS) modify competition among mtDNA haplotypes during oocyte development and/or at early embryonic stages. We show that heteroplasmy behavior can fall on a spectrum from random drift to strong selection, depending on mito-nuclear interactions and metabolic factors. Understanding heteroplasmy dynamics and its mechanisms provide novel knowledge of a fundamental biological process and enhance our ability to mitigate risks in clinical applications affecting mtDNA transmission.

Identifiants

pubmed: 31588014
pii: S1550-4131(19)30510-8
doi: 10.1016/j.cmet.2019.09.007
pmc: PMC6899444
pii:
doi:

Substances chimiques

DNA, Mitochondrial 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1120-1130.e5

Commentaires et corrections

Type : CommentIn

Informations de copyright

Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.

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Auteurs

Ana Latorre-Pellicer (A)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain; Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, ISS-Aragon, 50009 Zaragoza, Spain.

Ana Victoria Lechuga-Vieco (AV)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain; CIBERES: C/ Melchor Fernández-Almagro 3, 28029 Madrid, Spain.

Iain G Johnston (IG)

School of Biosciences, University of Birmingham, Birmingham, UK.

Riikka H Hämäläinen (RH)

Department of Neurobiology, A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio, Finland; Research Program of Molecular Neurology, Biomedicum, University of Helsinki, Helsinki, Finland.

Juan Pellico (J)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain; CIBERES: C/ Melchor Fernández-Almagro 3, 28029 Madrid, Spain.

Raquel Justo-Méndez (R)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Jose María Fernández-Toro (JM)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Cristina Clavería (C)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Adela Guaras (A)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Rocío Sierra (R)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Jordi Llop (J)

CIC biomaGUNE, Paseo Miramón No 182, San Sebastián, 20014 Guipúzcoa, Spain; IKERBASQUE, Basque Foundation for Science, Bilbao, Spain.

Miguel Torres (M)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Luis Miguel Criado (LM)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.

Anu Suomalainen (A)

Research Program of Molecular Neurology, Biomedicum, University of Helsinki, Helsinki, Finland.

Nick S Jones (NS)

Department of Mathematics, Imperial College London, London SW7 2BB, UK.

Jesús Ruíz-Cabello (J)

CIBERES: C/ Melchor Fernández-Almagro 3, 28029 Madrid, Spain; CIC biomaGUNE, Paseo Miramón No 182, San Sebastián, 20014 Guipúzcoa, Spain; IKERBASQUE, Basque Foundation for Science, Bilbao, Spain; Universidad Complutense de Madrid, Madrid 28606, Spain.

José Antonio Enríquez (JA)

Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain; CIBERFES: C/ Melchor Fernández-Almagro 3, 28029 Madrid, Spain. Electronic address: jaenriquez@cnic.es.

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Classifications MeSH