Human PPT1 Protein, His Tag
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产品编号
KMP3163
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别名
棕榈酰蛋白硫酯酶1, Human PPT1 Protein, His Tag, PPT1
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规格
- 50ug
- 100ug
- 200ug
Alias | 棕榈酰蛋白硫酯酶1, Human PPT1 Protein, His Tag, PPT1 |
Catalog Number | KMP3163 |
Product Description | The Human PPT1 Protein(KMP3163) is produced in HEK293 Cells. The target gene encoding the human PPT1 (P50897-1)(Met1-Gly306) is expressed with a C-terminal polyhistidine tag. |
Molecular Name | PPT1 |
Species | Human |
Host | HEK293 Cells |
Size | 50ug, 100ug, 200ug |
Purity | >90% as determined by SDS-PAGE |
Purification | Affinity purification |
Endotoxin | <1.0 EU/ug determined by the LAL method |
Formulation | PBS, pH7.4 |
Background | 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. |
Storage Condition | Aliquot and store at -20℃ to -80℃. Avoid repeated freezing and thawing cycles. |
Shipping Condition | In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. If supplied as liquid, the proteins will be shipped with dry ice. |
Molecular Weight | 32.7 kDa |