Imperial College London
Alternate

Nick S Jones

Faculty of Natural Sciences, Department of Mathematics

Professor of Mathematical Sciences
 
 
 
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Contact

 

+44 (0)20 7594 1146nick.jones

 
 
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Location

 

301aSir Ernst Chain BuildingSouth Kensington Campus

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Summary

 

Publications

Citation

BibTex format

@article{Burr:2023:10.1016/j.cell.2023.01.034,
author = {Burr, SP and Klimm, F and Glynos, A and Prater, M and Sendon, P and Nash, P and Powell, CA and Simard, M-L and Bonekamp, NA and Charl, J and Diaz, H and Bozhilova, LV and Nie, Y and Zhang, H and Frison, M and Falkenberg, M and Jones, N and Minczuk, M and Stewart, JB and Chinnery, PF},
doi = {10.1016/j.cell.2023.01.034},
journal = {Cell},
pages = {1212--1229.E21},
title = {Cell lineage-specific mitochondrial resilience during mammalian organogenesis},
url = {http://dx.doi.org/10.1016/j.cell.2023.01.034},
volume = {186},
year = {2023}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Mitochondrial activity differs markedly between organs, but it is not known how and when this arises. Here we show that cell lineage-specific expression profiles involving essential mitochondrial genes emerge at an early stage in mouse development, including tissue-specific isoforms present before organ formation. However, the nuclear transcriptional signatures were not independent of organelle function. Genetically disrupting intra-mitochondrial protein synthesis with two different mtDNA mutations induced cell lineage-specific compensatory responses, including molecular pathways not previously implicated in organellar maintenance. We saw downregulation of genes whose expression is known to exacerbate the effects of exogenous mitochondrial toxins, indicating a transcriptional adaptation to mitochondrial dysfunction during embryonic development. The compensatory pathways were both tissue and mutation specific and under the control of transcription factors which promote organelle resilience. These are likely to contribute to the tissue specificity which characterizes human mitochondrial diseases and are potential targets for organ-directed treatments.
AU  - Burr,SP
AU  - Klimm,F
AU  - Glynos,A
AU  - Prater,M
AU  - Sendon,P
AU  - Nash,P
AU  - Powell,CA
AU  - Simard,M-L
AU  - Bonekamp,NA
AU  - Charl,J
AU  - Diaz,H
AU  - Bozhilova,LV
AU  - Nie,Y
AU  - Zhang,H
AU  - Frison,M
AU  - Falkenberg,M
AU  - Jones,N
AU  - Minczuk,M
AU  - Stewart,JB
AU  - Chinnery,PF
DO  - 10.1016/j.cell.2023.01.034
EP  - 1229
PY  - 2023///
SN  - 0092-8674
SP  - 1212
TI  - Cell lineage-specific mitochondrial resilience during mammalian organogenesis
T2  - Cell
UR  - http://dx.doi.org/10.1016/j.cell.2023.01.034
UR  - https://www.ncbi.nlm.nih.gov/pubmed/36827974
UR  - https://www.cell.com/cell/fulltext/S0092-8674(23)00093-4?_returnURL=https%3A%2F%2Flinkinghub.elsevier.com%2Fretrieve%2Fpii%2FS0092867423000934%3Fshowall%3Dtrue
UR  - http://hdl.handle.net/10044/1/102870
VL  - 186
ER  -
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