Abcam, ab95950, Anti-FH/Fumarase antibody

Size: 50µL
Rabbit Polyclonal FH/Fumarase antibody. Suitable for ICC/IF, WB, IHC-P and reacts with Human, Mouse, Rat samples. Cited in 4 publications. Immunogen corresponding to Recombinant Fragment Protein within Human FH aa 50-350.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Rat, Human,
Applications:WB, IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human FH aa 50-350. The exact immunogen used to generate this antibody is proprietary information.P07954

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7Preservative: 0.01% Thimerosal (merthiolate)Constituents: 10% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Fumarase also known as fumarate hydratase (FH) catalyzes the reversible hydration of fumarate to malate in the citric acid cycle. This enzyme weighs approximately 49 kDa. Fumarase is predominantly expressed in the mitochondria where it performs its main function. However a cytosolic form exists that plays roles outside of the citric acid cycle especially related to DNA damage response.
Biological function summary
Fumarase converts fumarate into malate which is a critical step in cellular energy production. In the mitochondria fumarase acts as a part of the TCA cycle facilitating ATP generation. In the cytosol beyond its enzymatic activity fumarase participates in DNA repair indicating its dual functionality. This suggests that fumarase might play additional roles that are yet discovered.
Pathways
Fumarase plays essential roles in both the citric acid cycle and DNA repair pathways. In the TCA cycle fumarase along with enzymes like malate dehydrogenase maintains efficient energy conversion in mitochondria. In DNA repair fumarase likely interacts with proteins such as p53 suggesting that fumarate has signaling roles impacting cell survival and replication.
Mutations in the fumarase gene associate with hereditary leiomyomatosis and renal cell cancer (HLRCC). This disorder involves impaired fumarase function leading to abnormal cellular processes and tumor development. Fumarase deficiencies also connect to neurological disorders suggesting that its dysfunction has broad effects in cellular metabolism and genome stability linking it indirectly to other proteins within the affected pathways.