Fumarate Hydratase / FH Antibody

Fumarate Hydratase / FH Antibody Datasheet

Fumarate Hydratase / FH Antibody Product Information

• Immunogen: Recombinant Human Fumarate Hydratase / FH protein (Catalog#12115-H08E)
• Antibody Type: Mouse Monoclonal Antibody ( Mouse mAb Service Platform )
• Clone ID: 3D5A3G7
• Ig Type: Mouse IgG1
• Formulation: 0.2 μm filtered solution in PBS with 5% trehalose
• Preparation: This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human Fumarate Hydratase / FH (rh Fumarate Hydratase / FH; Catalog#12115-H08E; P07954-1; Ala 45-Lys 510). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.

Fumarate Hydratase / FH Antibody Usage Guide

• Specificity: Human Fumarate Hydratase / FH
• No cross-reactivity in ELISA with

  E.coli cell lysate

• Western blot: This antibody can be used at 1-2 μg/mL with the appropriate secondary reagents to detect Human FH in WB. Using a DAB detection system, the detection limit for Human FH is approximately 4 ng/lane under non-reducing conditions and 2 ng/lane under reducing conditions.
• Direct ELISA: This antibody can be used at 0.5-1 μg/mL with the appropriate secondary reagents to detect Human FH. The detection limit for Human FH is approximately 0.078 ng/well.
• Storage: This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -70℃. Preservative-Free.
  Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

Fumarate Hydratase / FH Antibody Background

Fumarate hydratase, also known as Fumarase and FH, is a protein which belongs to theclass-II fumarase / aspartase family and fumarase subfamily. The phenomenon of enhanced glycolysis in tumours has been acknowledged for decades, but biochemical evidence to explain it is only just beginning to emerge. Succinate dehydrogenase (SDH) and fumarate hydratase (FH) are tumour suppressors and which associated, mitochondrial enzymes and their dysfunction with tumorigenesis. Fumarate hydratase / FH immunohistochemistry may also serve as a useful tool to screen for hereditary leiomyomatosis and renal cell cancer (HLRCC) in young individuals with clear cell renal carcinoma. Defects in FH are the cause of fumarase deficiency (FHD) which also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. Defects in FH are also the cause of multiple cutaneous and uterine leiomyomata (MCUL1) which is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).

References

1. Phillips,T.M. et al., 2006,Pediatr Neurol. 35 (2): 150-3.
2. King,A. et al., 2006,Oncogene. 25 (34): 4675-82.
3. Barker,K.T. et al., 2006,Cancer Lett235 (1): 136-40.
4. Lehtonen,H.J. et al., 2007, Hum Pathol. 38 (5): 793-6.