A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice

A difference in the survival of respiratory chain complex III deficient Bcs1l p.S78G mice was observed between two congenic mouse strains. Here the authors show how in one of the strains the combined effects of a spontaneously arising non-pathogenic variant and the disease-causing Bcs1l p.S78G mutat...

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Autores principales:	Janne Purhonen, Vladislav Grigorjev, Robert Ekiert, Noora Aho, Jayasimman Rajendran, Rafał Pietras, Katarina Truvé, Mårten Wikström, Vivek Sharma, Artur Osyczka, Vineta Fellman, Jukka Kallijärvi
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2020
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/3779b9d3fc13415ab6b4e35cb40df347
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spelling	oai:doaj.org-article:3779b9d3fc13415ab6b4e35cb40df3472021-12-02T15:39:10ZA spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice10.1038/s41467-019-14201-22041-1723https://doaj.org/article/3779b9d3fc13415ab6b4e35cb40df3472020-01-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-14201-2https://doaj.org/toc/2041-1723A difference in the survival of respiratory chain complex III deficient Bcs1l p.S78G mice was observed between two congenic mouse strains. Here the authors show how in one of the strains the combined effects of a spontaneously arising non-pathogenic variant and the disease-causing Bcs1l p.S78G mutation exacerbate CIII deficiency and disease progression.Janne PurhonenVladislav GrigorjevRobert EkiertNoora AhoJayasimman RajendranRafał PietrasKatarina TruvéMårten WikströmVivek SharmaArtur OsyczkaVineta FellmanJukka KallijärviNature PortfolioarticleScienceQENNature Communications, Vol 11, Iss 1, Pp 1-12 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Janne Purhonen Vladislav Grigorjev Robert Ekiert Noora Aho Jayasimman Rajendran Rafał Pietras Katarina Truvé Mårten Wikström Vivek Sharma Artur Osyczka Vineta Fellman Jukka Kallijärvi A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
description	A difference in the survival of respiratory chain complex III deficient Bcs1l p.S78G mice was observed between two congenic mouse strains. Here the authors show how in one of the strains the combined effects of a spontaneously arising non-pathogenic variant and the disease-causing Bcs1l p.S78G mutation exacerbate CIII deficiency and disease progression.
format	article
author	Janne Purhonen Vladislav Grigorjev Robert Ekiert Noora Aho Jayasimman Rajendran Rafał Pietras Katarina Truvé Mårten Wikström Vivek Sharma Artur Osyczka Vineta Fellman Jukka Kallijärvi
author_facet	Janne Purhonen Vladislav Grigorjev Robert Ekiert Noora Aho Jayasimman Rajendran Rafał Pietras Katarina Truvé Mårten Wikström Vivek Sharma Artur Osyczka Vineta Fellman Jukka Kallijärvi
author_sort	Janne Purhonen
title	A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
title_short	A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
title_full	A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
title_fullStr	A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
title_full_unstemmed	A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice
title_sort	spontaneous mitonuclear epistasis converging on rieske fe-s protein exacerbates complex iii deficiency in mice
publisher	Nature Portfolio
publishDate	2020
url	https://doaj.org/article/3779b9d3fc13415ab6b4e35cb40df347
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A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice

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