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FH Enzyme

Fumarase, Fumarate Hydratase Mitochondrial, MCL, LRCC, HLRCC, MCUL1

Catalog No. Product Name Size List Price (US$) Quantity
BP000621-ENZ-395 Recombinant Human FH Enzyme 50 ug 160.00
BP000621-ENZ-395 Recombinant Human FH Enzyme 1 mg 1800.00
Description

Introduction
Fumarase is an enzymatic factor of Krebs cycle, which catalyzes the formation of L-malate from fumarate. Fumarase 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 aimed to the mitochondrion, where the removal of the extension results in the same form as in the cytoplasm. Fumarase is similar to a number of thermostable Class-2 fumarases and functions as a homotetramer. Mutations in the Fumarase gene causes fumarase deficiency and leads to progressive encephalopathy, cerebral atrophy and developmental delay. Fumarase enzyme is also thought to act as a tumor suppressor. Leydig cell tumors are caused by Fumarase mutations and represents one of the first reports of germline mutations in any type of adult testicular tumor.

BP000621-ENZ-395: Recombinant Human Fumarase (FH) Enzyme

Source: E. coli-derived.
A single, non-glycosylated polypeptide chain containing 467 amino acids (44-510) and having a molecular mass of 50.2 kDa.
Specific activity is > 1.0 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 25C.
Purity: > 95% by SDS-PAGE.
Formulation: The Fumarase protein solution (1 ug/ml) contains 20 mM Tris-HCl, pH-8.

Shipping: The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70°C as supplied.

Other FH Enzymes:
Recombinant Human FH Enzyme

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