| 产品名称 |
Recombinant Human FAH (C-6His) |
| 英文名称 |
Fumarylacetoacetase/FAH |
| 纯度 |
Greater than 95% as determined by reducing SDS-PAGE |
| 内毒素 |
<1 EU/µg as determined by LAL test. |
| 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. |
| Accession |
P16930 |
| 表达宿主 |
Human Cells |
| 种属 |
Human |
| 预测分子量 |
47.4 KDa |
| 制剂 |
Lyophilized from a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.5. |
| 运输方式 |
The product is shipped at ambient temperature.Upon receipt, store it immediately at the temperature listed below. |
| 稳定性&储存 |
Store at ≤-70°C, stable for 6 months after receipt.Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles. |
| 复溶 |
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. |
| 分子别名 |
| Fumarylacetoacetase; FAA; Beta-Diketonase; Fumarylacetoacetate Hydrolase; FAH |
| 背景介绍 |
| 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. |
注意事项
本司产品仅用于科研,不用于临床诊断和治疗