Abcam, ab110286, Anti-FH/Fumarase antibody [8F12BB5]

Size: 100µg
Mouse Monoclonal FH/Fumarase antibody. Suitable for IP, Flow Cyt, IHC-P, ICC/IF and reacts with Rat, Human, Cow samples. Cited in 2 publications.
Key facts
Host species:Mouse,
Clonality:Monoclonal,
Clone number:8F12BB5,
Isotype:IgG2a,
Light chain type:kappa,
Carrier free:No,
Reacts with:Rat, Cow, Human,
Applications:IHC-P, Flow Cyt, ICC/IF, IPSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:The exact immunogen used to generate this antibody is proprietary information.

Product details:
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Properties and Storage Information:
Form-Liquid, Purification technique-Precipitation Ammonium Sulphate, Purification notes-Purity near homogeneity as judged by SDS-PAGE. The antibody was produced in-vitro using hybridomas grown in serum-free medium and then purified by biochemical fractionation., Storage buffer-pH: 7.5Preservative: 0.02% Sodium azideConstituents: HEPES buffered saline, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Storage information-Do Not Freeze

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.