Fumarase/FH (clone AT3F6), anti-human

Description
Fumarase (Fumarate hytdratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid (TCA), or Krebs cycle. This enzyme exists in both a cytosolic form and an N-terminal extended mitochondrial form. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension is the same form as in the cytoplasm. Fumarase deficiency can lead to progressive encephalopathy, cerebral atrophy and developmental delay and this enzyme also is thought to act as a tumor suppressor.Alternative Names
Fumarate hydratase, FH, HLRCC, LRCC, MCL, MCuL1, Fumarate hydratase Fumarase, Fumarate hydratase mitochondrial, MCuL 1, Multiple hereditary cutaneous leiomyomata.Concentration
1mg/ml (determined by BCA assay)Concentration
Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerolStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.