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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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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1002/path.2932

Authors


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Institution:
University of Oxford
Division:
MSD
Department:
RDM
Sub department:
OCDEM
Role:
Author
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Institution:
University of Oxford
Division:
MSD
Department:
NDM
Sub department:
Jenner Institute
Role:
Author
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Publisher:
Wiley Publisher's website
Journal:
Journal of pathology Journal website
Volume:
225
Issue:
1
Pages:
4–11
Publication date:
2011-06-01
Acceptance date:
2011-05-03
DOI:
EISSN:
1096-9896
ISSN:
0022-3417
Source identifiers:
172466

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