|+1 404 474 4654|
|+1 888 205 9894 (TF)|
Fumarate Hydratase (FH) antibody
|Synonyms||FH, HF1, MGC140701, Fh1, zf-e152, MGC66253, zgc:66253, zgc:77498, ns:zf-e152, DKFZp469O0739, Fh-1, MCL, LRCC, HLRCC, MCUL1|
Alternatives ELISA, Immunofluorescence (IF), Western Blotting (WB)
|5 references available|
|Price||450.00 $ Plus shipping costs $45.00|
|Availability||Will be delivered in 2 to 3 Business Days|
|Immunogen||FH (AAH03108, 33 a.a. ~ 511 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 K|
|Description||Other names: HLRCC, LRCC, MCL, MCUL1 fumarate hydratase|
|Characteristics||Purified Mouse Monoclonal Antibody (Mab)|
|Specificity||FH (AAH03108, 33 a.a. ~ 511 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.|
|Molecular Weight||54637 DA|
Background: The protein encoded by this gene 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. It 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.
|Application Notes||ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000 Immunofluorescence|
|Buffer||Clear, colorless solution in phosphate buffered saline, pH 7.2 .|
|Storage||Maintain refrigerated at 2-8 deg C for up to 6 months. For long term storage store at -20 deg C in small aliquots to prevent freeze-thaw cycles|
|Research Area||Translation Factors, Signaling, Cell Structure|
|Restrictions||For Research Use only|
Tian, Liu, Usa et al.: "Novel role of fumarate metabolism in dahl-salt sensitive hypertension." in: Hypertension, Vol. 54, Issue 2, pp. 255-60, 2009 (PubMed).
McCauley, Zuvich, Bradford et al.: "Follow-up examination of linkage and association to chromosome 1q43 in multiple sclerosis." in: Genes and immunity, Vol. 10, Issue 7, pp. 624-30, 2009 (PubMed).
Yang, Valera, Padilla-Nash et al.: "UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer." in: Cancer genetics and cytogenetics, Vol. 196, Issue 1, pp. 45-55, 2009 (PubMed).
Yogev, Yogev, Singer et al.: "Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response." in: PLoS biology, Vol. 8, Issue 3, pp. e1000328, 2010 (PubMed).
Shimada, Miyagawa, Kawashima et al.: "An approach based on a genome-wide association study reveals candidate loci for narcolepsy." in: Human genetics, Vol. 128, Issue 4, pp. 433-41, 2010 (PubMed).