A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia

  title={A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia},
  author={Julie Adam and Ming Yang and Christina Bauerschmidt and Mitsuhiro Kitagawa and Linda O’Flaherty and Pratheesh Maheswaran and Gizem {\"O}zkan and Natasha Sahgal and Dilair F. Baban and Keiko Kato and Kaori Saito and Keiko Iino and Kaori Igarashi and Michael Stratford and Christopher W Pugh and Daniel A Tennant and Christian Ludwig and Benjamin Davies and Peter J Ratcliffe and Mona A. El-Bahrawy and Houman Ashrafian and Tomoyoshi Soga and Patrick J. Pollard},
  booktitle={Cell reports},
The identification of mutated metabolic enzymes in hereditary cancer syndromes has established a direct link between metabolic dysregulation and cancer. Mutations in the Krebs cycle enzyme, fumarate hydratase (FH), predispose affected individuals to leiomyomas, renal cysts, and cancers, though the respective pathogenic roles of mitochondrial and cytosolic FH isoforms remain undefined. On the basis of comprehensive metabolomic analyses, we demonstrate that FH1-deficient cells and tissues exhibit… CONTINUE READING
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