Fumarate Hydratase (FH) antibody

Antigen: Fumarate Hydratase (FH)

Synonyms: FH, HF1, MGC140701, Fh1, zf-e152, MGC66253, zgc:66253, zgc:77498, ns:zf-e152, DKFZp469O0739, Fh-1, MCL, LRCC, HLRCC, MCUL1

Clonality: Monoclonal (5C12)

Host: Mouse

Reactivity: Human

Conjugate: Un-conjugated

Application: ELISA, Immunofluorescence (IF), Western Blotting (WB)

Certificates: ISO 9001:2008

Catalog no.: ABIN394387

Additional Information

Alternative name: FH

Gene ID: 2271

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

Isotype: IgG2a kappa

Clone: 5C12

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

Comments:

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 Details

Application Notes: ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000 Immunofluorescence

Purification: Purified

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

Images

Immunofluorescence of monoclonal antibody to FH (AT2044a) on HeLa cell . [antibody concentration 10 ug/ml] FH monoclonal antibody (M07), clone 5C12 Western Blot analysis of FH expression in HeLa ( Cat # L013V1 ).

anti-Fumarate Hydratase (FH) antibody (Image 2)

Publications

Product: 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).