Cell Reports volume 3, issue 3, P689-700 2013 DOI: 10.1016/j.celrep.2013.02.013 View full text
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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 et al.

Abstract: SummaryThe 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 undertook a proteomic-based screen in cells and renal cysts from Fh1 (murine FH)-deficient mice and identified 94 protein succination targets. Notably, we identified the succination of three cysteine residues in…

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