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Human PPT1 Protein, His Tag

Human PPT1 Protein, His Tag

产品编号

KMP3163

别名

棕榈酰蛋白硫酯酶1, Human PPT1 Protein, His Tag, PPT1

规格
  • 50ug
  • 100ug
  • 200ug
产品介绍
Alias棕榈酰蛋白硫酯酶1, Human PPT1 Protein, His Tag, PPT1
Catalog NumberKMP3163
Product DescriptionThe 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 NamePPT1
SpeciesHuman
HostHEK293 Cells
Size50ug, 100ug, 200ug
Purity>90% as determined by SDS-PAGE
PurificationAffinity purification
Endotoxin<1.0 EU/ug determined by the LAL method
FormulationPBS, pH7.4
BackgroundMutations 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 ConditionAliquot and store at -20℃ to -80℃. Avoid repeated freezing and thawing cycles.
Shipping ConditionIn 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 Weight32.7 kDa

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