Human TPP1/CLN2 Recombinant Protein (RPES4815)
- SKU:
- RPES4815
- Product Type:
- Recombinant Protein
- Species:
- Human
Description
Human TPP1/CLN2 Recombinant Protein
Tripeptidyl-peptidase 1 (TPP1 / CLN2) is a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. TPP1 / CLN2 May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Defects in TPP1 / CLN2 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2), a form of neuronal ceroid lipofuscinosis which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy.
| Product Name: | Human TPP1/CLN2 Recombinant Protein (RPES4815) |
| Product Code: | RPES4815 |
| Size: | 10µg |
| Species: | Human |
| Expressed Host: | Baculovirus-Insect Cells |
| Synonyms: | Tripeptidyl-Peptidase 1, TPP-1, Cell Growth-Inhibiting Gene 1 Protein, Lysosomal Pepstatin-Insensitive Protease, LPIC, Tripeptidyl Aminopeptidase, TPP1, CLN2,GIG1,LPIC,SCAR7,TPP-1 |
| Accession: | AAH14863.1 |
| Sequence: | Met 1-Pro 563 |
| Fusion tag: | C-His |
| Activity: | Measured by the cleavage of AlaAlaPheAMC. The specific activity is > 850 pmoles/min/µg. |
| Endotoxin: | <1.0 EU per µg as determined by the LAL method. |
| Protein Construction: | A DNA sequence encoding the pro form of human TPP1 (AAH14863.1) (Met 1-Pro 563) was fused with a polyhistidine tag at the C-terminus. |
| Purity: | > 95 % as determined by reducing SDS-PAGE. |
| Mol Mass: | 60.7 kDa |
| AP Mol Mass: | 60 kDa |
| Formulation: | Lyophilized from sterile 20mM Tris, 500mM NaCl, pH 7.4, 10% gly |
| Shipping: | This product is provided as lyophilized powder which is shipped with ice packs. |
| Stability and Storage: | Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months. |
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