SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease
The pathomechanism of succinyl-CoA ligase (SCL) deficiency, a hereditary mitochondrial disease, is not fully understood. Here, the authors show that increased succinyl-CoA levels contribute to SCL pathology by causing global protein hyper-succinylation.
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Nature Portfolio
2020
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oai:doaj.org-article:21b100a7321c4480b27d69ad8980a8162021-12-02T14:16:47ZSUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease10.1038/s41467-020-19743-42041-1723https://doaj.org/article/21b100a7321c4480b27d69ad8980a8162020-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-020-19743-4https://doaj.org/toc/2041-1723The pathomechanism of succinyl-CoA ligase (SCL) deficiency, a hereditary mitochondrial disease, is not fully understood. Here, the authors show that increased succinyl-CoA levels contribute to SCL pathology by causing global protein hyper-succinylation.Philipp GutSanna MatilainenJesse G. MeyerPieti PällijeffJoy RichardChristopher J. CarrollLiliya EuroChristopher B. JacksonPirjo IsohanniBerge A. MinassianReem A. AlkhaterElsebet ØstergaardGabriele CivilettoAlice ParisiJonathan ThevenetMatthew J. RardinWenjuan HeYuya NishidaJohn C. NewmanXiaojing LiuStefan ChristenSofia MocoJason W. LocasaleBirgit SchillingAnu SuomalainenEric VerdinNature PortfolioarticleScienceQENNature Communications, Vol 11, Iss 1, Pp 1-14 (2020) |
| institution |
DOAJ |
| collection |
DOAJ |
| language |
EN |
| topic |
Science Q |
| spellingShingle |
Science Q Philipp Gut Sanna Matilainen Jesse G. Meyer Pieti Pällijeff Joy Richard Christopher J. Carroll Liliya Euro Christopher B. Jackson Pirjo Isohanni Berge A. Minassian Reem A. Alkhater Elsebet Østergaard Gabriele Civiletto Alice Parisi Jonathan Thevenet Matthew J. Rardin Wenjuan He Yuya Nishida John C. Newman Xiaojing Liu Stefan Christen Sofia Moco Jason W. Locasale Birgit Schilling Anu Suomalainen Eric Verdin SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| description |
The pathomechanism of succinyl-CoA ligase (SCL) deficiency, a hereditary mitochondrial disease, is not fully understood. Here, the authors show that increased succinyl-CoA levels contribute to SCL pathology by causing global protein hyper-succinylation. |
| format |
article |
| author |
Philipp Gut Sanna Matilainen Jesse G. Meyer Pieti Pällijeff Joy Richard Christopher J. Carroll Liliya Euro Christopher B. Jackson Pirjo Isohanni Berge A. Minassian Reem A. Alkhater Elsebet Østergaard Gabriele Civiletto Alice Parisi Jonathan Thevenet Matthew J. Rardin Wenjuan He Yuya Nishida John C. Newman Xiaojing Liu Stefan Christen Sofia Moco Jason W. Locasale Birgit Schilling Anu Suomalainen Eric Verdin |
| author_facet |
Philipp Gut Sanna Matilainen Jesse G. Meyer Pieti Pällijeff Joy Richard Christopher J. Carroll Liliya Euro Christopher B. Jackson Pirjo Isohanni Berge A. Minassian Reem A. Alkhater Elsebet Østergaard Gabriele Civiletto Alice Parisi Jonathan Thevenet Matthew J. Rardin Wenjuan He Yuya Nishida John C. Newman Xiaojing Liu Stefan Christen Sofia Moco Jason W. Locasale Birgit Schilling Anu Suomalainen Eric Verdin |
| author_sort |
Philipp Gut |
| title |
SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| title_short |
SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| title_full |
SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| title_fullStr |
SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| title_full_unstemmed |
SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| title_sort |
sucla2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease |
| publisher |
Nature Portfolio |
| publishDate |
2020 |
| url |
https://doaj.org/article/21b100a7321c4480b27d69ad8980a816 |
| work_keys_str_mv |
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