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Citation

BibTex format

@article{Lechuga-Vieco:2020:10.1126/sciadv.aba5345,
author = {Lechuga-Vieco, AV and Latorre-Pellicer, A and Johnston, IG and Prota, G and Gileadi, U and Justo-Méndez, R and Acín-Pérez, R and Martínez-de-Mena, R and Fernández-Toro, JM and Jimenez-Blasco, D and Mora, A and Nicolás-Ávila, JA and Santiago, DJ and Priori, SG and Bolaños, JP and Sabio, G and Criado, LM and Ruíz-Cabello, J and Cerundolo, V and Jones, NS and Enríquez, JA},
doi = {10.1126/sciadv.aba5345},
journal = {Science Advances},
pages = {eaba5345--eaba5345},
title = {Cell identity and nucleo-mitochondrial genetic context modulate OXPHOS performance and determine somatic heteroplasmy dynamics},
url = {http://dx.doi.org/10.1126/sciadv.aba5345},
volume = {6},
year = {2020}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Heteroplasmy, multiple variants of mitochondrial DNA (mtDNA) in the same cytoplasm, may be naturally generated by mutations but is counteracted by a genetic mtDNA bottleneck during oocyte development. Engineered heteroplasmic mice with nonpathological mtDNA variants reveal a nonrandom tissue-specific mtDNA segregation pattern, with few tissues that do not show segregation. The driving force for this dynamic complex pattern has remained unexplained for decades, challenging our understanding of this fundamental biological problem and hindering clinical planning for inherited diseases. Here, we demonstrate that the nonrandom mtDNA segregation is an intracellular process based on organelle selection. This cell type–specific decision arises jointly from the impact of mtDNA haplotypes on the oxidative phosphorylation (OXPHOS) system and the cell metabolic requirements and is strongly sensitive to the nuclear context and to environmental cues.
AU  - Lechuga-Vieco,AV
AU  - Latorre-Pellicer,A
AU  - Johnston,IG
AU  - Prota,G
AU  - Gileadi,U
AU  - Justo-Méndez,R
AU  - Acín-Pérez,R
AU  - Martínez-de-Mena,R
AU  - Fernández-Toro,JM
AU  - Jimenez-Blasco,D
AU  - Mora,A
AU  - Nicolás-Ávila,JA
AU  - Santiago,DJ
AU  - Priori,SG
AU  - Bolaños,JP
AU  - Sabio,G
AU  - Criado,LM
AU  - Ruíz-Cabello,J
AU  - Cerundolo,V
AU  - Jones,NS
AU  - Enríquez,JA
DO  - 10.1126/sciadv.aba5345
EP  - 5345
PY  - 2020///
SN  - 2375-2548
SP  - 5345
TI  - Cell identity and nucleo-mitochondrial genetic context modulate OXPHOS performance and determine somatic heteroplasmy dynamics
T2  - Science Advances
UR  - http://dx.doi.org/10.1126/sciadv.aba5345
UR  - https://advances.sciencemag.org/content/6/31/eaba5345
UR  - http://hdl.handle.net/10044/1/81586
VL  - 6
ER  -
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