Goat Anti-Fumarase (FH, fumarate hydratase, Fumarate hydratase, mitochondrial precursor, HLRCC, LRCC, MCL, MCUL1)
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.
Applications
Suitable for use in ELISA and Western Blot. Other applications not tested.
Recommended Dilutions
ELISA: 1:64,000 Western Blot: 0.01-0.03ug/ml Optimal dilutions to be determined by the researcher.
Positive Control
Human Kidney, Liver and Lung lysates
Storage and Stability
May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20°C. Aliquots are stable for at least 12 monthsat -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Peptide Blocking
Corresponding peptide is available for Peptide Blocking studies. See F9508-P. Antibody is typically 0.5mg/ml and peptide is supplied as a 100ul pellet. When peptide is reconstituted in 200ul water, the concentration would also be 0.5mg/ml. To start, the best ratio would be 1:1 (molar excess of peptide relative to antibody when identical volumes are mixed). Mix equal volumes of peptide and antibody at the required dilution and leave at ambient temperature. It is best is to have two identical blots to be incubated with equal amount of antibody, but one with the antibody pre-adsorbed to the peptide for 20min. Then incubate and develop the two blots in parallel.
Immunogen
Synthetic peptide, C-HPNDHVNKSQSSND, from the internal region of human Fumarase.
Form
Supplied as a liquid in Tris, pH 7.3, 0.5% BSA, 0.02% sodium azide.
Purity
Purified by affinity chromatography.
Specificity
Recognizes human Fumarase at ~48kD. Species sequence homology: mouse, rat, canine.
