Fumarate Hydratase Protein

Fumarate Hydratase Products

Fumarate Hydratase Protein, Recombinant

Molecule	Species	Description	Cat. No
Fumarate Hydratase	Human	Fumarate Hydratase Protein, Recombinant	12115-H08E

Fumarate Hydratase Antibody

Molecule	Application	Description	Cat. No
Human Fumarate Hydratase	WB, ELISA	Fumarate Hydratase / FH Antibody	12115-MM03
Human Fumarate Hydratase	WB, ELISA	Fumarate Hydratase / FH Antibody (Antigen Affinity Purified)	12115-RP02

Fumarate Hydratase cDNA Clone

Molecule	Species	Description	Cat. No
Fumarate Hydratase	Human	Homo sapiens FH/Fumarate Hydratase cDNA Clone	HG12115-M

Fumarate Hydratase Alternative Names

Fumarate Hydratase, FH, HLRCC, LRCC, MCL, MCUL1 [Homo sapiens]

Fumarate Hydratase, Fh1, Fh, Fh-1 [Mus musculus]

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.

Fumarate Hydratase Related Studies

1. King A, et al. (2005) Succinate dehydrogenase and fumarate hydratase: linking mitochondrial dysfunction and cancer. Oncogene. 25(34): 4675-82.
2. 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.
3. Pollard PJ, et al. (2003) The TCA cycle and tumorigenesis: the examples of fumarate hydratase and succinate dehydrogenase. Ann Med. 35(8): 632-9.