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| Catalog No. | Product Name | Size | List Price (US$) | Quantity |
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Conditions of optimal FAH Protein performance should be determined experimentally by the investigator.
Price/availability/specifications subject to change without notice. Unless otherwise indicated, our catalog and customized products are for research use only and not intended for human or animal diagnostic or therapeutic use.
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Fax: 1-617-606-5019
Email: message@sydlabs.com
Or leave a message with a formal purchase
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Introduction
Fumarylacetoacetase, also known as Fumarylacetoacetate hydrolase, Beta-diketonase, Fumarylacetoacetate hydrolase and FAH, belongs to the FAH family. Fumarylacetoacetase is primary expressed in liver and kidney. Fumarylacetoacetase exsit as a homodimer and catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. Defects in FAH are the cause of tyrosinemia type 1 which is an inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include renal tubular injury, self-mutilation,hepatic necrosis, episodic weakness and seizures.
BP001508-C874: Recombinant Human FAH (Fumarylacetoacetate hydrolase)
Source: HEK 293 ?erived.
Purity > 95%, by reducing SDS-PAGE and SEC-HPLC.
Endotoxin: Less than 0.1 ng/ug (1 EU/ug).
Shipping: The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70°C as supplied.
1 month, 2 to 8°C under sterile conditions after reconstitution.
3 months, -20 to -70°C under sterile conditions after reconstitution.
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