TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes

Mutations in the gene HADHA result in mitochondrial tri-functional protein (MTP) deficiency and can result in sudden infant death syndrome for which there is no treatment. Here the authors show that the MTP deficient pathology in human cardiomyocytes leads to an abnormal cardiolipin pattern and sugg...

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Autores principales:	Jason W. Miklas, Elisa Clark, Shiri Levy, Damien Detraux, Andrea Leonard, Kevin Beussman, Megan R. Showalter, Alec T. Smith, Peter Hofsteen, Xiulan Yang, Jesse Macadangdang, Tuula Manninen, Daniel Raftery, Anup Madan, Anu Suomalainen, Deok-Ho Kim, Charles E. Murry, Oliver Fiehn, Nathan J. Sniadecki, Yuliang Wang, Hannele Ruohola-Baker
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2019
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/eb9585b9475b4ec39e5021a1cc4c96fb
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spelling	oai:doaj.org-article:eb9585b9475b4ec39e5021a1cc4c96fb2021-12-02T17:01:58ZTFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes10.1038/s41467-019-12482-12041-1723https://doaj.org/article/eb9585b9475b4ec39e5021a1cc4c96fb2019-10-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-12482-1https://doaj.org/toc/2041-1723Mutations in the gene HADHA result in mitochondrial tri-functional protein (MTP) deficiency and can result in sudden infant death syndrome for which there is no treatment. Here the authors show that the MTP deficient pathology in human cardiomyocytes leads to an abnormal cardiolipin pattern and suggests that cardiolipin affecting compounds may serve as a potential therapy.Jason W. MiklasElisa ClarkShiri LevyDamien DetrauxAndrea LeonardKevin BeussmanMegan R. ShowalterAlec T. SmithPeter HofsteenXiulan YangJesse MacadangdangTuula ManninenDaniel RafteryAnup MadanAnu SuomalainenDeok-Ho KimCharles E. MurryOliver FiehnNathan J. SniadeckiYuliang WangHannele Ruohola-BakerNature PortfolioarticleScienceQENNature Communications, Vol 10, Iss 1, Pp 1-21 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Jason W. Miklas Elisa Clark Shiri Levy Damien Detraux Andrea Leonard Kevin Beussman Megan R. Showalter Alec T. Smith Peter Hofsteen Xiulan Yang Jesse Macadangdang Tuula Manninen Daniel Raftery Anup Madan Anu Suomalainen Deok-Ho Kim Charles E. Murry Oliver Fiehn Nathan J. Sniadecki Yuliang Wang Hannele Ruohola-Baker TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
description	Mutations in the gene HADHA result in mitochondrial tri-functional protein (MTP) deficiency and can result in sudden infant death syndrome for which there is no treatment. Here the authors show that the MTP deficient pathology in human cardiomyocytes leads to an abnormal cardiolipin pattern and suggests that cardiolipin affecting compounds may serve as a potential therapy.
format	article
author	Jason W. Miklas Elisa Clark Shiri Levy Damien Detraux Andrea Leonard Kevin Beussman Megan R. Showalter Alec T. Smith Peter Hofsteen Xiulan Yang Jesse Macadangdang Tuula Manninen Daniel Raftery Anup Madan Anu Suomalainen Deok-Ho Kim Charles E. Murry Oliver Fiehn Nathan J. Sniadecki Yuliang Wang Hannele Ruohola-Baker
author_facet	Jason W. Miklas Elisa Clark Shiri Levy Damien Detraux Andrea Leonard Kevin Beussman Megan R. Showalter Alec T. Smith Peter Hofsteen Xiulan Yang Jesse Macadangdang Tuula Manninen Daniel Raftery Anup Madan Anu Suomalainen Deok-Ho Kim Charles E. Murry Oliver Fiehn Nathan J. Sniadecki Yuliang Wang Hannele Ruohola-Baker
author_sort	Jason W. Miklas
title	TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
title_short	TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
title_full	TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
title_fullStr	TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
title_full_unstemmed	TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
title_sort	tfpa/hadha is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
publisher	Nature Portfolio
publishDate	2019
url	https://doaj.org/article/eb9585b9475b4ec39e5021a1cc4c96fb
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TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes

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