Fumarylacetoacetase/FAH Recombinant Protein (RPES4423)

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SKU:
RPES4423
Product type:
Recombinant Protein
Host species:
Human Cells
Reactivity:
Human
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Description

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Fumarylacetoacetase/FAH Recombinant Protein

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.

Product Name:Fumarylacetoacetase/FAH Recombinant Protein (RPES4423)
Product Code:RPES4423
Size:10µg
Species:Human
Expressed Host:Human Cells
Synonyms:Fumarylacetoacetase, FAA, Beta-Diketonase, Fumarylacetoacetate Hydrolase, FAH
Accession:P16930
Sequence:Ser2-Ser419
Fusion tag:C-6His
Endotoxin:<1.0 EU per µg as determined by the LAL method.
Protein 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.
Purity:> 95 % as determined by reducing SDS-PAGE.
Mol Mass:47.4 kDa
AP Mol Mass:47 kDa
Formulation:Lyophilized from a 0.2 µm filtered solution of PBS, pH7.4.
Shipping:This product is provided as lyophilized powder which is shipped with ice packs.
Stability and Storage:Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
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