FH rabbit pAb

• 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]

• Description :

  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],

• Product Name Alternative :

  Fumarate hydratase, mitochondrial (Fumarase) (EC 4.2.1.2)

• UniProt :

  P07954

• Swiss Prot :

  P07954

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

  Recombinant Protein of Fumarate hydratase, mitochondrial

• Target :

  FH

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB

• Dilution :

  WB: 1:1000

• Buffer :

  -20°C/1 year

• Molecular Weight :

  50kD

• Storage Conditions :

  -20°C/1 year

• Observed Molecular Weight :

  50kD

• Fragment :

  IgG

• 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 Product Names :

  Fumarate hydratase, mitochondrial (Fumarase) (EC 4.2.1.2)

• Gene ID (Human) :

  2271