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C874

Recombinant Human Fumarylacetoacetase/FAH

10ug

840

756

现货

国产

C874

Recombinant Human Fumarylacetoacetase/FAH

50ug

2520

2268

现货

国产

C874

Recombinant Human Fumarylacetoacetase/FAH

500ug

12320

11088

现货

国产

C874

Recombinant Human Fumarylacetoacetase/FAH

1mg

17600

15840

现货

国产

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  • Catalog# C874
    Source Human Cells
    Description Recombinant Human Fumarylacetoacetase/FAH is produced by our mammalian expression system in human cells. The target protein is expressed with sequence (Ser2-Ser419) of Human FAH fused with a polyhistidine tag at the C-terminus.
    Names Fumarylacetoacetase, FAA, Beta-Diketonase, Fumarylacetoacetate Hydrolase, FAH
    Accession # P16930
    Formulation Lyophilized from a 0.2 μm filtered solution of PBS,pH7.4
    Shipping The product is shipped at ambient temperature.
    Reconstitution 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 1X PBS.
    Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
    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.
    Purity Greater than 95% as determined by SEC-HPLC and reducing SDS-PAGE.
    Endotoxin Less than 0.1 ng/µg (1 IEU/µg).
    Amino Acid Sequence
    SFIPVAEDSDFPIHNLPYGVFSTRGDPRPRIGVAIGDQILDLSIIKHLFTGPVLSKHQDVFNQPT LNSFMGLGQAAWKEARVFLQNLLSVSQARLRDDTELRKCAFISQASATMHLPATIGDYTDFYSSR QHATNVGIMFRDKENALMPNWLHLPVGYHGRASSVVVSGTPIRRPMGQMKPDDSKPPVYGACKLL DMELEMAFFVGPGNRLGEPIPISKAHEHIFGMVLMNDWSARDIQKWEYVPLGPFLGKSFGTTVSP WVVPMDALMPFAVPNPKQDPRPLPYLCHDEPYTFDINLSVNLKGEGMSQAATICKSNFKYMYWTM LQQLTHHSVNGCNLRPGDLLASGTISGPEPENFGSMLELSWKGTKPIDLGNGQTRKFLLDGDEVI ITGYCQGDGYRIGFGQCAGKVLPALLPSVDHHHHHH
    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.