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Two transgenic mouse models for β-subunit components of succinate-CoA ligase yielding pleiotropic metabolic alterations

Succinate-CoA ligase (SUCL) is a heterodimer enzyme composed of Suclg1 α-subunit and a substrate-specific Sucla2 or Suclg2 β-subunit yielding ATP or GTP, respectively. In humans, the deficiency of this enzyme leads to encephalomyopathy with or without methylmalonyl aciduria, in addition to resulting...

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Detalles Bibliográficos
Autores principales: Kacso, Gergely, Ravasz, Dora, Doczi, Judit, Németh, Beáta, Madgar, Ory, Saada, Ann, Ilin, Polina, Miller, Chaya, Ostergaard, Elsebet, Iordanov, Iordan, Adams, Daniel, Vargedo, Zsuzsanna, Araki, Masatake, Araki, Kimi, Nakahara, Mai, Ito, Haruka, Gál, Aniko, Molnár, Mária J., Nagy, Zsolt, Patocs, Attila, Adam-Vizi, Vera, Chinopoulos, Christos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Portland Press Ltd. 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5126846/
https://www.ncbi.nlm.nih.gov/pubmed/27496549
http://dx.doi.org/10.1042/BCJ20160594