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Description
Fumarate hydratase Recombinant Rabbit mAb (SDT-2434-54)Product Specification Host Rabbit Antigen Fumarate hydratase Synonyms Fumarate hydratase, mitochondrial; Fumarase; HsFH; FH Immunogen Synthetic Peptide Location Cytoplasm, Nucleus, Mitochondrion Accession P07954 Clone Number SDT 2434 54 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P, ICC Reactivity Hu, Ms, Rt Positive Sample HeLa, HEK 293, HepG2, MCF7, NIH 3T3, PC 12 Purification Protein A Concentration 0. 5 mg ml Conjugation
Product Specification
| Host | Rabbit |
| Antigen | Fumarate hydratase |
| Synonyms | Fumarate hydratase, mitochondrial; Fumarase; HsFH; FH |
| Immunogen | Synthetic Peptide |
| Location | Cytoplasm, Nucleus, Mitochondrion |
| Accession | P07954 |
| Clone Number | SDT-2434-54 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB, IHC-P, ICC |
| Reactivity | Hu, Ms, Rt |
| Positive Sample | HeLa, HEK-293, HepG2, MCF7, NIH/3T3, PC-12 |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000-1:2000 | Hu, Ms, Rt |
| IHC-P | 1:1000 | Hu |
| ICC | 1:500 | Hu |
Background
Fumarate hydratase (FH) is a mitochondrial and cytoplasmic metabolic enzyme encoded by the FH gene that catalyzes the reversible stereospecific hydration of fumarate to L-malate within the tricarboxylic acid cycle, a reaction essential for NADH production and cellular energy homeostasis; beyond this canonical metabolic role, FH acts as a tumor suppressor because loss-of-function mutations lead to intracellular accumulation of the oncometabolite fumarate, which inhibits α-ketoglutarate-dependent dioxygenases (including HIF prolyl-hydroxylases and histone demethylases), thereby stabilizing HIF-1α, driving pseudohypoxic gene expression, succinating KEAP1 to activate Nrf2 antioxidant signaling, and ultimately promoting hereditary leiomyomatosis and renal cell carcinoma, while complete FH deficiency causes the severe neurometabolic disorder fumaric aciduria characterized by encephalopathy, developmental delay, and elevated fumarate levels, and the enzyme also moonlights in DNA repair by translocating to double-strand breaks where locally generated fumarate enhances non-homologous end-joining through inhibition of KDM2B and increased H3K36 dimethylation.
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