FH rabbit pAb

SKU: ES20754

Size: 100μL

Source:Rabbit

Applications:WB

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],