Recombinant human Fumarase/FH protein
Fumarase (Fumarate hytdratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid (TCA), or Krebs cycle. This enzyme exists in both a cytosolic form and an N-terminal extended 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 deficiency can lead to progressive encephalopathy, cerebral atrophy and developmental delay and this enzyme also is thought to act as a tumor suppressor. Recombinant Fumarase was expressed in E. coli and was purified by conventional chromatography techniques.
Product Specifications
Specifications
Specific activity is > 25 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 37C.
Product Name Alternative
Expression System
E.coli
Antigen Species
Human
Tag
Non-Tagged
Applications
SDS-PAGE, Enzyme Activity
Concentration
1 mg/mL (determined by Bradford assay)
Endotoxin
< 1 EU per 1ug of protein (determined by LAL method)
Purity
> 95% by SDS-PAGE
Bioactivity
Specific activity is > 25 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 37C.
Molecular Weight
50.2 kDa (467aa) confirmed by MALDI-TOF
Additionnal Information
References & Citations
Lehtonen R., et al. (2003) J. Med. Genet. 40 (3) : e19.; ; Toro JR., et al. (2003) Am. J. Hum. Genet. 73 (1) : 95-106.;
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0)
Scientific Category
Cell Cycle
NCBI Accession Number
NP_000134
Uniprot Accession Number
P07954
Species
Human
AA Sequence
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