产品描述 概述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. 使用说明This material is offered by Novin Biotech for research, laboratory or further evaluation purposes. NOT FOR HUMAN USE. 技术规格| Tag | C-6His | | 种属 | Human | | 表达系统 | Human cells | | Accession# | P16930 | | Source | Human Cells | | Formulation_Description | Lyophilized from a 0.2 μm filtered solution of 20mM Tris, 150mM NaCl, pH 8.5. | | Storage | Lyophilized protein should be stored at < -20°C, though stable at room temperature for 3 weeks.Reconstituted protein solution can be stored at 4-7°C for 2-7 days.Aliquots of reconstituted samples are stable at < -20°C for 3 months. | | Reconstitution | Dissolve the lyophilized protein in distilled water. | | Purity | Greater than 95% as determined by reducing SDS-PAGE. | | Endotoxin | Less than 0.1 ng/μg (1 EU/μg) as determined by LAL test. | | Background | 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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