Article
Two transgenic mouse models for β-subunit components of succinate-CoA ligase yielding pleiotropic metabolic alterations.
The Biochemical journal - 15 Oct 2016
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
Abstract excerpt
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 in mitochondrial DNA depletion. We generated mice lacking either one Sucla2 or Suclg2 allele. Sucla2 heterozygote...
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