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Description
Fumarate hydratase Rabbit Polyclonal AntibodyProduct Specification Host Rabbit Antigen Fumarate hydratase Synonyms Fumarate hydratase, mitochondrial; Fumarase; HsFH; FH Immunogen Synthetic Peptide Location Mitochondrion Accession P07954 Antibody Type Polyclonal antibody Isotype IgG Application WB, IHC P, ICC Reactivity Hu, Ms Positive Sample HeLa, NIH 3T3 Purification Immunogen Affinity Concentration 0. 5 mg ml Conjugation Unconjugated Physical Appearance Liquid Storage Buffer PBS, 40% Glycerol,
Product Specification
| Host | Rabbit |
| Antigen | Fumarate hydratase |
| Synonyms | Fumarate hydratase, mitochondrial; Fumarase; HsFH; FH |
| Immunogen | Synthetic Peptide |
| Location | Mitochondrion |
| Accession | P07954 |
| Antibody Type | Polyclonal antibody |
| Isotype | IgG |
| Application | WB, IHC-P, ICC |
| Reactivity | Hu, Ms |
| Positive Sample | HeLa, NIH/3T3 |
| Purification | Immunogen Affinity |
| 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 | Hu, Ms |
| IHC-P | 1:1000 | Hu, Ms |
| ICC | 1:500 | Ms |
Background
Fumarate hydratase (FH) is a crucial enzyme encoded by the FH gene located on chromosome 1q42.3-q43, and it exists as a homotetramer. This enzyme catalyzes the reversible conversion of fumarate to L-malate in the tricarboxylic acid (TCA) cycle, playing a key role in cellular metabolism and energy production. FH has two forms: a mitochondrial form involved in the TCA cycle and a cytosolic form that participates in amino acid metabolism and DNA damage repair. Germline mutations in the FH gene are associated with hereditary leiomyomatosis and renal cell carcinoma (HLRCC), a syndrome characterized by an increased risk of developing leiomyomas in the skin and uterus, as well as an aggressive form of renal cancer. The loss of FH function leads to the accumulation of fumarate, which stabilizes HIF1α and promotes tumorigenesis through the upregulation of HIF-target genes.
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