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Aberrant succination of proteins in fumarate hydratase-deficient mice and HLRCC patients is a robust biomarker of mutation status.

Abstract:

Germline mutations in the FH gene encoding the Krebs cycle enzyme fumarate hydratase predispose to hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome. FH-deficient cells and tissues accumulate high levels of fumarate, which may act as an oncometabolite and contribute to tumourigenesis. A recently proposed role for fumarate in the covalent modification of cysteine residues to S-(2-succinyl) cysteine (2SC) (termed protein succination) prompted us to assess 2SC levels in our existi...

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Publisher copy:
10.1002/path.2932

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Journal:
J Pathol
Volume:
225
Issue:
1
Pages:
4-11
Publication date:
2011-09-05
DOI:
EISSN:
1096-9896
ISSN:
0022-3417
URN:
uuid:5ab23cbc-aa2c-4260-b2e6-0f885ce1027c
Source identifiers:
140312
Local pid:
pubs:140312

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