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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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| Main Authors: | , , , , , , , , , , , , , , , , |
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| פורמט: | Artigo |
| שפה: | Inglês |
| יצא לאור: |
Cell Press
2013
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| גישה מקוונת: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3617368/ https://ncbi.nlm.nih.gov/pubmed/23499446 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2013.02.013 |
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