Recombinant Human Fumarylacetoacetase/FAH (C-6His)

Recombinant Human Fumarylacetoacetase/FAH (C-6His)

Catalog Number:
P001411268ABE
Mfr. No.:
Abe32-7683
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      • Overview
        • MW : 47.4kD. 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. 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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      • Properties
        • Source
          Human cells
          Type
          Recombinant Proteins
          Formulation
          Lyophilized from a 0.2 µm filtered solution of PBS.
          Storage
          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.
          Endotoxin
          Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test.

          More Information

          UniProt
          Gene ID
          2184

          * For Research Use Only. Not for use in diagnostic/therapeutics procedures.

      • Applications
        • Application Description
          Always centrifuge tubes before opening. Do not mix by vortex or pipetting. 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.

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