PPT1 Protein, Human, Recombinant (hFc Tag)

• 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.

• Synonyms:

  CLN1 Protein, Human; INCL Protein, Human; PPT Protein, Human; PPT1 Protein, Human

• Gene Name:

  Palmitoyl-protein thioesterase 1

• UniProt:

  P50897-1

• Expression System:

  HEK293 Cells

• Tag:

  C-hFc

• Endotoxin:

  < 1.0 EU per μg of the protein as determined by the LAL method.

• Purity:

  > 85 % as determined by SDS-PAGE.

• Activity:

  At Leading Biology, the biological activity of a recombinant protein is routinely measured using a bioassay, e.g. chemotaxis or cell proliferation assay, enzyme assay, or a functional ELISA. When a recombinant protein is an enzyme, specific activity is derived from an enzymatic assay. Each enzyme is tested for potency by cleavage of a substrate. We are in the process of updating the biological activity data. If you have any questions regarding this update, please feel free to contact our technical support team.

• Form:

  Lyophilized

• Buffer:

  Lyophilized from sterile PBS, pH 7.4. Please contact us for any concerns or special requirements. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.

• Molecular Weight:

  The recombinant human PPT1/Fc comprises 520 amino acids and has a predicted molecular mass of 58.3 kDa. The apparent molecular mass of the protein is approximately 58-67 kDa in SDS-PAGE under reducing conditions.

• Storage Conditions:

  In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise. Stable for 1 year at -20°C or below from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and opening the cap. For long-term storage, aliquot and store at -20°C or below. Avoid repeated freeze-thaw cycles.

• Symbol:

  PPT1

• Species:

  Human

• Overview:

  A DNA sequence encoding the human PPT1 (P50897-1) (Met1-Gly306) was expressed with the Fc region of human IgG1 at the C-terminus.

• Uniprot URL:

  https://www.uniprot.org/uniprot/P50897-1

• AA Sequence:

  Met1-Gly306