| Name | Recombinant Human FAH (C-6His) |
| Purity | Greater than 95% as determined by reducing SDS-PAGE |
| Endotoxin level | <1 EU/µg as determined by LAL test. |
| Construction | 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. |
| Accession # | P16930 |
| Host | Human Cells |
| Species | Human |
| Predicted Molecular Mass | 47.4 KDa |
| Buffer | Lyophilized from a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.5. |
| Form | Lyophilized |
| Shipping | The product is shipped at ambient temperature.Upon receipt, store it immediately at the temperature listed below. |
| Stability&Storage | Store at ≤-70°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. |
| 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. |
Alternative Names
Fumarylacetoacetase; FAA; Beta-Diketonase; Fumarylacetoacetate Hydrolase; FAH
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.
Note
For Research Use Only , Not for Diagnostic Use.