Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan

Changes in the rate and fidelity of mitochondrial protein synthesis impact the metabolic and physiological roles of mitochondria. Here we explored how environmental stress in the form of a high-fat diet modulates mitochondrial translation and affects lifespan in mutant mice with error-prone (Mrps12e...

Full description

Bibliographic Details
Main Authors: Richman, T.R., Ermer, J.A., Siira, S.J., Kuznetsova, I., Brosnan, C.A., Rossetti, G., Baker, J., Perks, K.L., Cserne Szappanos, H., Viola, H.M., Gray, N., Larance, M., Hool, L.C., Zuryn, S., Rackham, Oliver, Filipovska, A.
Format: Journal Article
Language:English
Published: WILEY 2021
Subjects:
Online Access:http://purl.org/au-research/grants/arc/DP180101656
http://hdl.handle.net/20.500.11937/90821
_version_ 1848765437966286848
author Richman, T.R.
Ermer, J.A.
Siira, S.J.
Kuznetsova, I.
Brosnan, C.A.
Rossetti, G.
Baker, J.
Perks, K.L.
Cserne Szappanos, H.
Viola, H.M.
Gray, N.
Larance, M.
Hool, L.C.
Zuryn, S.
Rackham, Oliver
Filipovska, A.
author_facet Richman, T.R.
Ermer, J.A.
Siira, S.J.
Kuznetsova, I.
Brosnan, C.A.
Rossetti, G.
Baker, J.
Perks, K.L.
Cserne Szappanos, H.
Viola, H.M.
Gray, N.
Larance, M.
Hool, L.C.
Zuryn, S.
Rackham, Oliver
Filipovska, A.
author_sort Richman, T.R.
building Curtin Institutional Repository
collection Online Access
description Changes in the rate and fidelity of mitochondrial protein synthesis impact the metabolic and physiological roles of mitochondria. Here we explored how environmental stress in the form of a high-fat diet modulates mitochondrial translation and affects lifespan in mutant mice with error-prone (Mrps12ep/ep) or hyper-accurate (Mrps12ha/ha) mitochondrial ribosomes. Intriguingly, although both mutations are metabolically beneficial in reducing body weight, decreasing circulating insulin and increasing glucose tolerance during a high-fat diet, they manifest divergent (either deleterious or beneficial) outcomes in a tissue-specific manner. In two distinct organs that are commonly affected by the metabolic disease, the heart and the liver, Mrps12ep/ep mice were protected against heart defects but sensitive towards lipid accumulation in the liver, activating genes involved in steroid and amino acid metabolism. In contrast, enhanced translational accuracy in Mrps12ha/ha mice protected the liver from a high-fat diet through activation of liver proliferation programs, but enhanced the development of severe hypertrophic cardiomyopathy and led to reduced lifespan. These findings reflect the complex transcriptional and cell signalling responses that differ between post-mitotic (heart) and highly proliferative (liver) tissues. We show trade-offs between the rate and fidelity of mitochondrial protein synthesis dictate tissue-specific outcomes due to commonly encountered stressful environmental conditions or aging.
first_indexed 2025-11-14T11:35:15Z
format Journal Article
id curtin-20.500.11937-90821
institution Curtin University Malaysia
institution_category Local University
language English
last_indexed 2025-11-14T11:35:15Z
publishDate 2021
publisher WILEY
recordtype eprints
repository_type Digital Repository
spelling curtin-20.500.11937-908212023-04-24T01:19:05Z Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan Richman, T.R. Ermer, J.A. Siira, S.J. Kuznetsova, I. Brosnan, C.A. Rossetti, G. Baker, J. Perks, K.L. Cserne Szappanos, H. Viola, H.M. Gray, N. Larance, M. Hool, L.C. Zuryn, S. Rackham, Oliver Filipovska, A. Science & Technology Life Sciences & Biomedicine Cell Biology Geriatrics & Gerontology ageing metabolism mitochondria protein synthesis SIRT3 Changes in the rate and fidelity of mitochondrial protein synthesis impact the metabolic and physiological roles of mitochondria. Here we explored how environmental stress in the form of a high-fat diet modulates mitochondrial translation and affects lifespan in mutant mice with error-prone (Mrps12ep/ep) or hyper-accurate (Mrps12ha/ha) mitochondrial ribosomes. Intriguingly, although both mutations are metabolically beneficial in reducing body weight, decreasing circulating insulin and increasing glucose tolerance during a high-fat diet, they manifest divergent (either deleterious or beneficial) outcomes in a tissue-specific manner. In two distinct organs that are commonly affected by the metabolic disease, the heart and the liver, Mrps12ep/ep mice were protected against heart defects but sensitive towards lipid accumulation in the liver, activating genes involved in steroid and amino acid metabolism. In contrast, enhanced translational accuracy in Mrps12ha/ha mice protected the liver from a high-fat diet through activation of liver proliferation programs, but enhanced the development of severe hypertrophic cardiomyopathy and led to reduced lifespan. These findings reflect the complex transcriptional and cell signalling responses that differ between post-mitotic (heart) and highly proliferative (liver) tissues. We show trade-offs between the rate and fidelity of mitochondrial protein synthesis dictate tissue-specific outcomes due to commonly encountered stressful environmental conditions or aging. 2021 Journal Article http://hdl.handle.net/20.500.11937/90821 10.1111/acel.13408 English http://purl.org/au-research/grants/arc/DP180101656 http://creativecommons.org/licenses/by/4.0/ WILEY fulltext
spellingShingle Science & Technology
Life Sciences & Biomedicine
Cell Biology
Geriatrics & Gerontology
ageing
metabolism
mitochondria
protein synthesis
SIRT3
Richman, T.R.
Ermer, J.A.
Siira, S.J.
Kuznetsova, I.
Brosnan, C.A.
Rossetti, G.
Baker, J.
Perks, K.L.
Cserne Szappanos, H.
Viola, H.M.
Gray, N.
Larance, M.
Hool, L.C.
Zuryn, S.
Rackham, Oliver
Filipovska, A.
Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title_full Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title_fullStr Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title_full_unstemmed Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title_short Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
title_sort mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan
topic Science & Technology
Life Sciences & Biomedicine
Cell Biology
Geriatrics & Gerontology
ageing
metabolism
mitochondria
protein synthesis
SIRT3
url http://purl.org/au-research/grants/arc/DP180101656
http://hdl.handle.net/20.500.11937/90821