FH rabbit pAb
ENT-A16008
Description
| REF | ENT-A16008 |
|---|---|
| Category | Antibody Polyclonal |
| Description | FH rabbit pAb |
| Source | Rabbit |
| Applications | WB |
| Reactivity | Human;Mouse;Rat |
| Reactivity | Human;Mouse;Rat |
| Dilution | WB: 1:1000 |
| Immunogen | Recombinant Protein of Fumarate hydratase, mitochondrial |
| Storage Stability | -20°C/1 year |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | |
| Observed Band KD | 50kD |
| Human Gene ID | 2271 |
| Human Swiss Prot Nº | P07954 |
| Subcellular Location | [Isoform Mitochondrial]: Mitochondrion .; [Isoform Cytoplasmic]: Cytoplasm, cytosol . Nucleus . Chromosome . Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs) following phosphorylation by PRKDC. . |
Other Name: Fumarate hydratase, mitochondrial (Fumarase) (EC 4.2.1.2)
Background: The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008],