BackgroundThe 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.
DescriptionFH Polyclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
SpecificityHuman, Mouse, Rat
IsotypeIgG
Uniprot IDP07954
PurificationAffinity Purification
ImmunogenRecombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human FH (NP_000134.2).
StorageShipped at 4°C. Upon receipt, store at -20°C. Avoid freeze / thaw cycles
Alternative NamesFH; FMRD; HLRCC; LRCC; MCL; MCUL1; fumarate hydratase
ApplicationWB, IHC, IF