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Contributor Information

  • Name Eyal Gottlieb
  • Institute Cancer Research UK, Glasgow: The Beatson Institute

Tool Details

  • Tool name: FH-/- CL 1 Cell Line
  • Alternate names: Fumarate hydratase, FH, fumarase, Fh1, Hereditary leiomyomatosis and renal cell cancer, HLRCC
  • Tool type: Cell Lines
  • Organism: Mouse
  • Tissue: Kidney
  • Disease: Cancer
  • Model: Knock-Out
  • Conditional: No
  • Description: Fumarate hydratase which can be found in either a mitochondrial or cytosolic form - is an enzyme that catalyzes the reversible hydration and dehydration of fumarate to malate. Signal sequences located in the protein dictate the subcellular location of each FH isoenzyme. The cytosolic form is involved in the metabolism of amino acids and fumarate and the mitochondrial form is involved in the Krebs Cycle (tricarboxylic acid cycle or the citric acid cycle). Germline mutations of FH are responsible for hereditary leiomyomatosis and renal-cell cancer (HLRCC)1.
  • Research area: Cancer; Drug Discovery & Development; Metabolism
  • Cellosaurus ID: CVCL_HG01

  • For Research Use Only

Target Details

  • Target: Fumarate Hydratase

Application Details

Handling

  • Format: Frozen
  • Storage conditions: Liquid Nitrogen
  • Shipping conditions: Dry ice
  • Mycoplasma free: Yes
  • Biosafety level: 1

Documentation

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  • Available on request

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References

  • •  Frezza et al. 2011. Nature. 477(7363):225-8. PMID: 21849978.
  • •  Haem oxygenase is synthetically lethal with the tumour suppressor fumarate hydratase.
  • •  Pollard et al. 2007. Cancer Cell. 11(4):311-9. PMID: 17418408.
  • •  Targeted inactivation of fh1 causes proliferative renal cyst development and activation of the hypoxia pathway.