Recombinant Human Fumarylacetoacetase/FAH is produced by our mammalian expression system in human cells. The target protein is expressed with sequence (Ser2-Ser419) of Human FAH fused with a polyhistidine tag at the C-terminus.
Pureté
> 95 % as determined by reducing SDS-PAGE.
Stérilité
0.2 μm filtered
niveau d'endotoxine
Less than 0.1 ng/μg (1 IEU/μg) as determined by LAL test
FAH
Origine: Rat
Hôte: Levure
Recombinant
> 90 %
ELISA
Restrictions
For Research Use only
Format
Lyophilized
Reconstitution
It is not recommended to reconstitute to a concentration less than 100 μg/mL. Dissolve the lyophilized protein in ddH2O. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
Buffer
Lyophilized from a 0.2 μm filtered solution of PBS.
Conseil sur la manipulation
Always centrifuge tubes before opening. Do not mix by vortex or pipetting.
Stock
4 °C/-20 °C/-80 °C
Stockage commentaire
Lyophilized protein should be stored at < -20°C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 4-7°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
Fumarylacetoacetase belongs to the FAH family. Fumarylacetoacetase is primary expressed in liver and kidney. It exists as a homodimer and catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. Defects in Fumarylacetoacetase cause tyrosinemia type 1, which is congenital metabolism defect characterized by elevated levels of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include renal tubular injury, self-mutilation, hepatic necrosis, episodic weakness, and seizures. Alternative Names: Fumarylacetoacetase, FAA, Beta-Diketonase, Fumarylacetoacetate Hydrolase, FAH