FH-/- CL 19 Cell Line

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.

Contributors

Inventor Institute
Eyal Gottlieb Cancer Research UK, Glasgow: The Beatson Institute
Cat. #: 153290
Unit size: 1×10^6 cells / vial
Research Fields: Cancer;Drug development;Metabolism
Organism: Mouse
Tissue: Kidney
Model: Knock-Out
Alternate name: Fumarate hydratase, FH, fumarase, Fh1, Hereditary leiomyomatosis and renal cell cancer, HLRCC
Product 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 responsi…
Cellosaurus ID: CVCL_HG02
disease: Cancer
Format: Frozen
Storage conditions: Liquid Nitrogen
Shipping conditions: Dry ice
Growth medium: Cells can be maintained in DMEM supplemented with 10% FBS, 2 mmol/l glutamine, 1 mmol/l pyruvate and 50 mg/l uridine. However, the inventor advises to use more physiological conditions for experiments, such as Plasmax, available on our website.
Mycoplasma free: Yes
Biosafety level: 1
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.

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