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Reactivity | Human Mouse Rat |
Tested applications | WB IHC IF |
Recommended Dilution | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:10 - 1:100 |
Calculated MW | 54kDa |
Observed MW | Refer to Figures |
Immunogen | Recombinant protein of human FH |
Storage Buffer | Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Synonym | MCL; LRCC; HLRCC; 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.
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