| Catalog# | 
            C224 | 
        
        
            | Source | 
            E.coli | 
        
        
            | Description | 
            Recombinant Human Fumarase/FH is produced by our E. coli expression system. The target protein is expressed with sequence (Ala45-Lys510) of Human FH. | 
        
        
            | Names | 
            Fumarate Hydratase Mitochondrial, Fumarase, FH | 
        
        
            | Accession # | 
            P07954 | 
        
        
            | Formulation | 
            Supplied as a 0.2 μm filtered solution of 20mM Tris, pH 8.0 | 
        
        
            | Shipping | 
            The product is shipped on dry ice/ice packs. | 
        
        
            | Storage | 
            Store at < -20°C, stable for 6 months after receipt. 
            Please minimize freeze-thaw cycles. | 
        
        
            | Purity | 
            Greater than 95% as determined by reducing SDS-PAGE. | 
        
        
            | Endotoxin | 
            Less than 0.1 ng/μg (1 IEU/μg). | 
        
        
            | Amino Acid Sequence | 
            
             MASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQ DYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKI PVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQD AVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVAAKVAALTGL PFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPRSGLGELILPENEPGSSIM PGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSARLLGDASVSFTEN CVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELGYLTAEQFD EWVKPKDMLGPK 
             | 
        
        
            | Background | 
            Fumarase is an enzyme that catalyze the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid or Krebs cycle. Fumarase exists in both form, cytosolic form and N-terminal extend mitochondrial form. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension is the same form as in the cytoplasm. Fumarase is thought to act as a tumor suppressor, which deficiency can lead to progressive encephalopathy, cerebral atrophy and development delay. |