Rabbit polyclonal primary
FH Antibody (ER1902-01)
Recombinant protein within human fh aa 50-300.
K562 cell lysates, LOVO, SiHa, rat testis tissue, human liver tissue, human liver carcinoma tissue, mouse colon tissue, HCT116.
Store at +4C after thawing. Aliquot store at -20C. Avoid repeated freeze / thaw cycles.
1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.
Protein affinity purified.
FH antibody; Fumarase antibody; Fumarate hydratase antibody; Fumarate hydratase mitochondrial antibody; Fumarate hydratase, mitochondrial antibody; FUMH_HUMAN antibody; HLRCC antibody; LRCC antibody; MCL antibody; MCUL 1 antibody; MCUL1 antibody; MS709 antibody; Multiple hereditary cutaneous leiomyomata antibody
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.
[Isoform Cytoplasmic]: Phosphorylation at Thr-236 by PRKDC in response to DNA damage promotes translocation to the nucleus and recruitment to DNA double-strand breaks (DSBs).
Mitochondrion, cytosol, nucleus, chromosome.
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.