FH/Fumarate Hydratase Proteins, Antibodies, cDNA Clones Research Reagents

All FH/Fumarate Hydratase reagents are produced in house and quality controlled, including 4 FH/Fumarate Hydratase Antibody, 30 FH/Fumarate Hydratase Gene, 1 FH/Fumarate Hydratase IP Kit, 1 FH/Fumarate Hydratase Protein, 1 FH/Fumarate Hydratase qPCR. All FH/Fumarate Hydratase reagents are ready to use.

FH/Fumarate Hydratase Protein (1)

    FH/Fumarate Hydratase Antibody (4)

      FH/Fumarate Hydratase cDNA Clone (30)

      FH/Fumarate Hydratase qPCR Primer (1)

      FH/Fumarate Hydratase Background

      Fumarate Hydratase (FH) is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. Fumarate Hydratase is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. Individuals with hemizygous germline fumarate hydratase (FH) mutations are predisposed to renal cancer. These tumors predominantly exhibit functional inactivation of the remaining wild-type allele, implicating FH inactivation as a tumor-promoting event.

      FH/Fumarate Hydratase References

      • King A, et al. (2005) Succinate dehydrogenase and fumarate hydratase: linking mitochondrial dysfunction and cancer. Oncogene. 25(34): 4675-82.
      • Alam NA, et al. (2003) Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency. Hum Mol Genet.12(11): 1241-52.
      • Pollard PJ, et al. (2003) The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenase. Ann Med. 35(8): 632-9.

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