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Fumarase (FH, fumarate hydratase, Fumarate hydratase, mitochondrial precursor, HLRCC, LRCC, MCL, MCUL1)

Fumarase (FH, fumarate hydratase, Fumarate hydratase, mitochondrial precursor, HLRCC, LRCC, MCL, MCUL1)

Cat no: F9508

Supplier: United States Biological
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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 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 monthsat -20 degrees 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
Catalogue number: F9508
Reactivities: Human
Hosts: Goat
Applications: ELISA, Western Blot
Size: 100ug
Form: Supplied as a liquid in Tris, pH 7.3, 0.5% BSA, 0.02% sodium azide.
P type: Pab
Isotype: IgG
Purity: Purified by affinity chromatography.
References: Pollard PJ, Spencer-Dene B, Shukla D, Howarth K, Nye E, El-Bahrawy M, Deheragoda M, Joannou M, McDonald S, Martin A, Igarashi P, Varsani-Brown S, Rosewell I, Poulsom R, Maxwell P, Stamp GW, omlinson IP. Targeted inactivation of fh1 causes proliferative renal cyst development and activation of the hypoxia pathway. Cancer Cell. 2007 Apr;11(4):311-9.
Additional info: Recognizes human Fumarase at ~48kD. Species sequence homology: mouse, rat, canine.

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