Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

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 un...

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Bibliographic Details
Main Authors: Nicola Ternette, Ming Yang, Mahima Laroyia, Mitsuhiro Kitagawa, Linda O’Flaherty, Kathryn Wolhulter, Kaori Igarashi, Kaori Saito, Keiko Kato, Roman Fischer, Alexandre Berquand, Benedikt M. Kessler, Terry Lappin, Norma Frizzell, Tomoyoshi Soga, Julie Adam, Patrick J. Pollard
Format: Article
Language:English
Published: Elsevier 2013-03-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124713000727

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http://www.sciencedirect.com/science/article/pii/S2211124713000727

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