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Human Fumarate Hydratase / FH Protein (His Tag)

FMRD,HLRCC,LRCC,MCL,MCUL1

Catalog Number P12115-H08E
Organism Species Human
Host E. coli
Synonyms FMRD,HLRCC,LRCC,MCL,MCUL1
Molecular Weight The recombinant human FH consisting of 477 amino acids and has a calculated molecular mass of 52 kDa. It migrates as an approximately 45 kDa band in SDS-PAGE under reducing conditions.
predicted N Met
SDS-PAGE
Purity > 85 % as determined by SDS-PAGE
Protein Construction A DNA sequence encoding the mature form of human FH (P07954-1) (Ala 45-Lys 510) was fused with a polyhistidine tag at the C-terminus and an initial Met at the N-terminus.
Bio-activity
Research Area Cancer |Oncoprotein & suppressor & biomarker |Tumor biomarker |Tumor Suppressor
Formulation Lyophilized from sterile 10mM Tris, 5mM EDTA, 1mM DTT, pH 7.5
1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
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.
Reference
  • 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.