Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome
Leigh syndrome (LS) is a severe neurometabolic disorder which lacks effective models. Here, the authors developed human neuronal models of LS carrying mutations in SURF1 which show impaired neuronal morphogenesis due to metabolic deficiencies.
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Nature Portfolio
2021
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oai:doaj.org-article:7c5258ec4a0c4d51b4034db5722f87a12021-12-02T16:35:53ZDefective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome10.1038/s41467-021-22117-z2041-1723https://doaj.org/article/7c5258ec4a0c4d51b4034db5722f87a12021-03-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-22117-zhttps://doaj.org/toc/2041-1723Leigh syndrome (LS) is a severe neurometabolic disorder which lacks effective models. Here, the authors developed human neuronal models of LS carrying mutations in SURF1 which show impaired neuronal morphogenesis due to metabolic deficiencies.Gizem InakAgnieszka Rybak-WolfPawel LisowskiTancredi M. PentimalliRené JüttnerPetar GlažarKaran UppalEmanuela BottaniDario BrunettiChristopher SeckerAnnika ZinkDavid MeierhoferMarie-Thérèse HenkeMonishita DeyUmmi CiptasariBarbara MlodyTobias HahnMaria Berruezo-LlacunaNikos KaraiskosMichela Di VirgilioJohannes A. MayrSaskia B. WortmannJosef PrillerMichael GotthardtDean P. JonesErtan MayatepekWerner StenzelSebastian DieckeRalf KühnErich E. WankerNikolaus RajewskyMarkus SchuelkeAlessandro PrigioneNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-22 (2021) |
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DOAJ |
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DOAJ |
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EN |
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Science Q |
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Science Q Gizem Inak Agnieszka Rybak-Wolf Pawel Lisowski Tancredi M. Pentimalli René Jüttner Petar Glažar Karan Uppal Emanuela Bottani Dario Brunetti Christopher Secker Annika Zink David Meierhofer Marie-Thérèse Henke Monishita Dey Ummi Ciptasari Barbara Mlody Tobias Hahn Maria Berruezo-Llacuna Nikos Karaiskos Michela Di Virgilio Johannes A. Mayr Saskia B. Wortmann Josef Priller Michael Gotthardt Dean P. Jones Ertan Mayatepek Werner Stenzel Sebastian Diecke Ralf Kühn Erich E. Wanker Nikolaus Rajewsky Markus Schuelke Alessandro Prigione Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| description |
Leigh syndrome (LS) is a severe neurometabolic disorder which lacks effective models. Here, the authors developed human neuronal models of LS carrying mutations in SURF1 which show impaired neuronal morphogenesis due to metabolic deficiencies. |
| format |
article |
| author |
Gizem Inak Agnieszka Rybak-Wolf Pawel Lisowski Tancredi M. Pentimalli René Jüttner Petar Glažar Karan Uppal Emanuela Bottani Dario Brunetti Christopher Secker Annika Zink David Meierhofer Marie-Thérèse Henke Monishita Dey Ummi Ciptasari Barbara Mlody Tobias Hahn Maria Berruezo-Llacuna Nikos Karaiskos Michela Di Virgilio Johannes A. Mayr Saskia B. Wortmann Josef Priller Michael Gotthardt Dean P. Jones Ertan Mayatepek Werner Stenzel Sebastian Diecke Ralf Kühn Erich E. Wanker Nikolaus Rajewsky Markus Schuelke Alessandro Prigione |
| author_facet |
Gizem Inak Agnieszka Rybak-Wolf Pawel Lisowski Tancredi M. Pentimalli René Jüttner Petar Glažar Karan Uppal Emanuela Bottani Dario Brunetti Christopher Secker Annika Zink David Meierhofer Marie-Thérèse Henke Monishita Dey Ummi Ciptasari Barbara Mlody Tobias Hahn Maria Berruezo-Llacuna Nikos Karaiskos Michela Di Virgilio Johannes A. Mayr Saskia B. Wortmann Josef Priller Michael Gotthardt Dean P. Jones Ertan Mayatepek Werner Stenzel Sebastian Diecke Ralf Kühn Erich E. Wanker Nikolaus Rajewsky Markus Schuelke Alessandro Prigione |
| author_sort |
Gizem Inak |
| title |
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| title_short |
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| title_full |
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| title_fullStr |
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| title_full_unstemmed |
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome |
| title_sort |
defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of leigh syndrome |
| publisher |
Nature Portfolio |
| publishDate |
2021 |
| url |
https://doaj.org/article/7c5258ec4a0c4d51b4034db5722f87a1 |
| work_keys_str_mv |
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