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Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

Abstract:

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 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 ...

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Journal:
Cell Reports
Volume:
3
Issue:
3
Pages:
689-700
Publication date:
2013-01-01
DOI:
EISSN:
2211-1247
URN:
uuid:141d1929-2159-4c46-8d3b-45303a9b7d39
Source identifiers:
396037
Local pid:
pubs:396037

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