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.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
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 |
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 | Human Cells |
标签 | C-6His |
蛋白编号 | P16930 |
别名 | Fumarylacetoacetate Hydrolase, β-Diketonase, FAH, FAA, Fumarylacetoacetase, Beta-Diketonase |
氨基酸序列 | Ser2-Ser419 |
蛋白构建 | 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. |
蛋白纯度 | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 43 KDa, reducing conditions |
内毒素 | Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
缓冲液 | Lyophilized from a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.5. |
复溶方法 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
存储 |
Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
运输方式 |
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature listed below. |
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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FAH Protein, Human, Recombinant (His) Fumarylacetoacetate Hydrolase β-Diketonase b-Diketonase FAH FAA Fumarylacetoacetase Beta-Diketonase recombinant recombinant-proteins proteins protein