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SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease

Mitochondrial acyl-coenzyme A species are emerging as important sources of protein modification and damage. Succinyl-CoA ligase (SCL) deficiency causes a mitochondrial encephalomyopathy of unknown pathomechanism. Here, we show that succinyl-CoA accumulates in cells derived from patients with recessi...

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Detalhes bibliográficos
Publicado no:Nat Commun
Main Authors: Gut, Philipp, Matilainen, Sanna, Meyer, Jesse G., Pällijeff, Pieti, Richard, Joy, Carroll, Christopher J., Euro, Liliya, Jackson, Christopher B., Isohanni, Pirjo, Minassian, Berge A., Alkhater, Reem A., Østergaard, Elsebet, Civiletto, Gabriele, Parisi, Alice, Thevenet, Jonathan, Rardin, Matthew J., He, Wenjuan, Nishida, Yuya, Newman, John C., Liu, Xiaojing, Christen, Stefan, Moco, Sofia, Locasale, Jason W., Schilling, Birgit, Suomalainen, Anu, Verdin, Eric
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group UK 2020
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7684291/
https://ncbi.nlm.nih.gov/pubmed/33230181
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41467-020-19743-4
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