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FAH Protein, Human, Recombinant (His)

(Synonyms: β-Diketonase, Fumarylacetoacetate Hydrolase, Fumarylacetoacetase, FAH, FAA, Beta-Diketonase) Copy Product Info

Synonyms: β-Diketonase, Fumarylacetoacetate Hydrolase, Fumarylacetoacetase, FAH, FAA, Beta-Diketonase

Catalog No. TMPJ-00876 Copy Product Info
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.
FAH Protein, Human, Recombinant (His)
Pack SizePriceUSA StockGlobal StockQuantity
5 μg$11235 days35 days
10 μg$18335 days35 days
20 μg$29235 days35 days
50 μg$54535 days35 days
100 μg$79235 days35 days
200 μg$1,15035 days35 days
500 μg$1,90035 days35 days
1 mg$2,73035 days35 days
For In stock only · Estimated delivery: USA Stock (1-2 days) Global Stock (5-7 days)
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For research use only—not for human use. No sales to individuals. Use as intended only.
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Product Introduction

Bioactivity
Bioactivity
Activity has not been tested. It is theoretically active, but we cannot guarantee it. If you require protein activity, we recommend choosing the eukaryotic expression version first.
Description
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.
Species
Human
Expression System
HEK293 Cells
TagC-6xHis
Accession NumberP16930
Amino AcidSer2-Ser419
ConstructionSer2-Ser419
Protein Purity
Greater than 95% as determined by reducing SDS-PAGE. (QC verified)
Endotoxin< 0.1 ng/µg (1 EU/µg) as determined by LAL test.
FormulationLyophilized from a solution filtered through a 0.22 μm filter, containing 20 mM Tris-HCl, 150 mM NaCl, pH 8.5.
ReconstitutionReconstitute the lyophilized protein in distilled water. The product concentration should not be less than 100 μg/ml. Before opening, centrifuge the tube to collect powder at the bottom. After adding the reconstitution buffer, avoid vortexing or pipetting for mixing.
Stability & StorageLyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at -80°C. For reconstituted protein solutions, the solution can be stored at -20°C to -80°C for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots.
ShippingIn general, lyophilized powders are shipped with blue ice, while solutions are shipped with dry ice.
Synonymsβ-Diketonase, Fumarylacetoacetate Hydrolase, Fumarylacetoacetase, FAH, FAA, Beta-Diketonase
Research Background
Chemical Properties
Molecular Weight43 KDa (reducing condition)
Storage & Solubility Information
StorageLyophilized powder: -20~-80°C for 1 year | Solution: -80°C for 6 months

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Tech Support

Please see Inhibitor Handling Instructions for more frequently ask questions. Topics include: how to prepare stock solutions, how to store products, and cautions on cell-based assays & animal experiments, etc

Keywords

Related Tags: FAH Protein, Human, Recombinant (His) chemical structure | FAH Protein, Human, Recombinant (His) molecular weight