Description
Product Name: | FH Polyclonal Antibody |
Product Code: | CAB21403 |
Reactivity: | Human, Mouse, Rat |
Applications: | Western blotting |
Host Species: | Rabbit |
Purification Method: | Affinity purification |
Isotype: | IgG |
Reactivity: | Human, Mouse, Rat |
Tested Applications: | WB, ELISA |
Key Applications: | Western blotting |
Recommended Dilution: | WB 1:500-1:1000 |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Positive Samples: | 293T, Hela, NIH/3T3, C2C12, Rat liver |
Cellular Location: | Cytoplasm, Mitochondrion |
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.
Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 50-150 of human FH (NP_000134.2). |
Sequence: | FRIEY DTFGE LKVPN DKYYG AQTVR STMNF KIGGV TERMP TPVIK AFGIL KRAAA EVNQD YGLDP KIANA IMKAA DEVAE GKLND HFPLV VWQTG SGTQT N |
Synonyms: | MCL, FMRD, HsFH, LRCC, HLRCC, MCUL1 |
Calculated MW: | 55kDa |
Observed MW: | 49kDa |