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Human Recombinant Fumarylacetoacetase (from Cells)
Human Recombinant Fumarylacetoacetase (from Cells)
# de catalogue 75789-996
Fournisseur:  Prosci
Numéro CAS:  
Human Recombinant Fumarylacetoacetase (from Cells)
# de catalogue 75789-996
Fournisseur:  Prosci
Numéro CAS:  
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Spécifications

  • Enzyme type:
    Recombinant
  • Source:
    Cells
  • Species:
    Human
  • Size:
    0.05 mg
  • Storage conditions:
    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 content:
    <0.1 ng/ug (1 IEU/ug) as determined by LAL test.
  • Enzyme name:
    Fumarylacetoacetase
  • Enzyme synonyms:
    Fumarylacetoacetase, FAA, Beta-Diketonase, Fumarylacetoacetate Hydrolase, FAH
  • Purity:
    >95% as determined by reducing SDS-PAGE
  • Molecular weight:
    47.4 kD
  • Sequence:
    Ser2-Ser419
  • Formulation:
    Lyophilized from a 0.2 µm filtered solution of PBS. Always centrifuge tubes before opening. Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100 ug/ml. Dissolve the Lyophilized protein in ddH2O. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
  • Tested applications:
    Bioassay
  • Cat. no.:
    75789-996
  • Supplier No.:
    91-668

Spécifications

A propos de cet article

Fumarylacetoacetase belongs to the FAH family

This recombinant protein can be used for biological assays. For research use only.

Fusion-Tag: C-6 His tag

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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