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 und...

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Bibliographic Details
Main Authors: 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.
Format: Online
Language:English
Published: Cell Press 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3617368/

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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3617368/

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