Recombinant Human FAH (C-6His)

Product Specifications

Background

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.

Product Name Alternative

Fumarylacetoacetase; FAA; Beta-Diketonase; Fumarylacetoacetate Hydrolase; FAH

Accession Number

P16930

Host

Human Cells

Endotoxin

<1 EU/µg as determined by LAL test.

Purity

Greater than 95% as determined by reducing SDS-PAGE

Form

Lyophilized

Reconstitution

Always centrifuge tubes before opening.Do not mix by vortex or pipetting.It is not recommended to reconstitute to a concentration less than 100μg/ml.Dissolve the lyophilized protein in distilled water.Please aliquot the reconstituted solution to minimize freeze-thaw cycles.

Molecular Weight

47.4 KDa

Shipping Conditions

The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature listed below.

Storage Conditions

Store at -70°C/-20°C, stable for 6 months after receipt.Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles.

Formulation

Lyophilized from a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.5.

Construct

Recombinant Human Fumarylacetoacetase is produced by our Mammalian expression system and the target gene encoding Ser2-Ser419 is expressed with a 6His tag at the C-terminus.