Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

The gene encoding the Krebs cycle enzyme fumarate hydratase (FH) is mutated in hereditary leiomyomatosis and renal cell cancer (HLRCC). Loss of FH activity causes accumulation of intracellular fumarate, which can directly modify cysteine residues to form 2-succinocysteine through succination. We und...

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Главные авторы: Ternette, Nicola, Yang, Ming, Laroyia, Mahima, Kitagawa, Mitsuhiro, O’Flaherty, Linda, Wolhulter, Kathryn, Igarashi, Kaori, Saito, Kaori, Kato, Keiko, Fischer, Roman, Berquand, Alexandre, Kessler, Benedikt M., Lappin, Terry, Frizzell, Norma, Soga, Tomoyoshi, Adam, Julie, Pollard, Patrick J.
Формат: Artigo
Язык:Inglês
Опубликовано: Cell Press 2013
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Report
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC3617368/
https://ncbi.nlm.nih.gov/pubmed/23499446
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2013.02.013
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