FAH Protein, Human, Recombinant (His)

产品编号 TMPJ-00876

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)

产品编号 TMPJ-00876

规格	价格	库存
10 μg	¥ 1,170	5日内发货
50 μg	¥ 3,470	5日内发货
500 μg	¥ 12,100	5日内发货
1 mg	¥ 17,400	5日内发货

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TargetMol 的所有产品仅用作科学研究或药证申报，不能被用于人体，我们不向个人提供产品和服务。请您遵守承诺用途，不得违反法律法规规定用于任何其他用途。

产品信息

生物活性	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.
产品描述	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.
种属	Human
表达系统	HEK293 Cells
标签	C-6xHis
蛋白编号	P16930
别名	β-Diketonase,Fumarylacetoacetate Hydrolase,Fumarylacetoacetase,FAH,FAA,Beta-Diketonase
氨基酸序列	Ser2-Ser419
蛋白构建	Ser2-Ser419
蛋白纯度	Greater than 95% as determined by reducing SDS-PAGE. (QC verified)
分子量	43 KDa (reducing condition)
内毒素	< 0.1 ng/µg (1 EU/µg) as determined by LAL test.
缓冲液	Lyophilized from a solution filtered through a 0.22 μm filter, containing 20 mM Tris-HCl, 150 mM NaCl, pH 8.5.
复溶方法	Reconstitute 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.
存储	Lyophilized 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.
运输方式	In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice.
研究背景	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.

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关键词

b-Diketonase