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ELK Biotechnology

SKU:ES20754

FH rabbit pAb

FH rabbit pAb

Regular price $250.00 USD
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Size
Applications: WB
Reactivity: Human;Mouse;Rat
Source: Rabbit
Dilution: WB: 1:1000
Immunogen: Recombinant Protein of Fumarate hydratase, mitochondrial
Storage_stability: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
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],
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