PPT1 Protein, Human, Recombinant (hFc) is expressed in HEK293 mammalian cells with hFc tag. The predicted molecular weight is 58.3 kDa and the accession number is P50897-1.
生物活性 | Testing in progress |
产品描述 | PPT1 Protein, Human, Recombinant (hFc) is expressed in HEK293 mammalian cells with hFc tag. The predicted molecular weight is 58.3 kDa and the accession number is P50897-1. |
种属 | Human |
表达系统 | HEK293 Cells |
标签 | C-hFc |
蛋白编号 | P50897-1 |
别名 | INCL, PPT1, PPT, palmitoyl-protein thioesterase 1, CLN1 |
蛋白构建 | The Human PPT1 (P50897-1)(Met1-Gly306) was expressed with the Fc region of Human IgG1 at the C-terminus. |
蛋白纯度 | > 85 % as determined by SDS-PAGE |
分子量 | 58.3 kDa (predicted) |
内毒素 | < 1.0 EU/μg of the protein as determined by the LAL method. |
缓冲液 | Lyophilized from a solution filtered through a 0.22 μm filter, containing PBS, pH 7.4. Typically, a mixture containing 5% to 8% trehalose, mannitol, and 0.01% Tween 80 is incorporated as a protective agent before lyophilization. |
复溶方法 | A Certificate of Analysis (CoA) containing reconstitution instructions is included with the products. Please refer to the CoA for detailed information. |
存储 |
It is recommended to store recombinant proteins at -20°C to -80°C for future use. Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, 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. |
研究背景 | Mutations in the depalmitoylating enzyme gene, PPT1, cause the infantile form of Neuronal Ceroid Lipofuscinosis (NCL), an early onset neurodegenerative disease. Mutations in palmitoyl protein thioesterase-1 (PPT1) have been found to cause the infantile form of neuronal ceroid lipofuscinosis, which is a lysosomal storage disorder characterized by impaired degradation of fatty acid-modified proteins with accumulation of amorphous granular deposits in cortical neurons, leading to mental retardation and death. PPT1 catalyzes the cleavage of thioester linkages in S-acylated (palmitoylated) proteins and its deficiency leads to abnormal accumulation of thioesterified polypeptides (ceroid) in lysosomes causing INCL pathogenesis. |
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PPT1 Protein, Human, Recombinant (hFc) INCL CLN 1 PPT 1 PPT1 CLN-1 PPT PPT-1 palmitoyl-protein thioesterase 1 CLN1 recombinant recombinant-proteins proteins protein