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.1) Shimada, M., et al. Hum. Genet. 128(4):433-441(2010)
Immunogen
This FH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 107-135 amino acids from the N-terminal region of human FH.
Western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the detected the FH protein (arrow).
Application Notes
For WB starting dilution is: 1:1000
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.