Applications
-
WB
-
ICC
-
IHC-P
-
FC
REACTIVITY
-
Human
-
Mouse
-
Rat
SPECIFICATIONS
Product Type
Rabbit polyclonal primary
Product Name
FH Rabbit Polyclonal Antibody (ER1902-01)
Immunogen
Recombinant protein within human fh aa 83-267 / 510.
Host
Rabbit
Positive Control
K562 cell lysates, LOVO, SiHa, rat testis tissue, human liver tissue, human liver carcinoma tissue, mouse colon tissue, HCT116.
Conjugation
Unconjugated
Clonality
Polyclonal
PROPERTIES
Form
Liquid
Storage Condition
Store at +4C after thawing. Aliquot store at -20C. Avoid repeated freeze / thaw cycles.
Storage Buffer
1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.
Concentration
1 ug/ul
PURIFICATION
Protein affinity purified.
MOLECULAR WEIGHT
55 kDa
Isotype
IgG
APPLICATION DILUTION
-
WB:1:500-1:1,000
-
ICC:1:50-1:100
-
IHC-P:1:50-1:200
-
FC:1:50-1:100
TARGET
UNIPROT #
PROTEIN NAME
FH
SYNONYMS
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
SEQUENCE SIMILARITIES
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
TISSUE SPECIFICITY
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.
POST-TRANSLATIONAL MODIFICATION
[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).
SUBCELLULAR LOCATION
Mitochondrion, cytosol, nucleus, chromosome.
FUNCTION
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.