Name: Human Fumarylacetoacetase/FAH Recombinant Protein (His Tag)
Synonyms: Fumarylacetoacetase;FAA;Beta-Diketonase;Fumarylacetoacetate Hydrolase;FAH
Expression host: HEK293 Cells
Sequence: Ser2-Ser419
Accesstion: P16930
Species: Human
Mol_Mass: 47.4 kDa
AP_Mol_Mass: 43 kDa
Tag: C-His
Purity: > 95 % as determined by reducing SDS-PAGE.
Endotoxin:
Storage: Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at
Shipping: This product is provided as lyophilized powder which is shipped with ice packs.
Formulation: Lyophilized from a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.5.Normally 5 % – 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.Please refer to the specific buffer information in the print
Reconstitution: Please refer to the printed manual for detailed information.
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.
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