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|Tested species reactivity||Human|
|Host / Isotype||Goat / IgG|
|Immunogen||Synthetic peptide sequence (HPNDHVNKSQSSND) corresponding to the internal amino acids of FH|
|Purification||Antigen affinity chromatography|
|Storage buffer||TBS, pH 7.3, with 0.5% BSA|
|Contains||0.02% sodium azide|
|Storage Conditions||-20° C, Avoid Freeze/Thaw Cycles|
|Tested Applications||Dilution *|
|ELISA (ELISA)||1-2 ug/ml|
|Western Blot (WB)||0.01-0.03 ug/ml|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
This antibody is predicted to react with canine, mouse and rat based on sequence homology.
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Fumarase; fumarate hydratase; Fumarate hydratase, mitochondrial; HLRCC; leiomyomatosis and renal cell cancer; LRCC; MCL; MCUL1; multiple hereditary cutaneous leiomyomata
FH; FMRD; HLRCC; LRCC; MCL; MCUL1