| Description : |
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. |
| Source : |
E. coli |
| Species : |
Human |
| Tag : |
His |
| Form : |
Lyophilized powder/frozen liquid |
| Molecular Mass : |
36.69 kDa |
| Protein Length : |
Met1-Val320 |
| Purity : |
> 90 % as determined by SDS-PAGE |
| Notes : |
For research use only. |
| Storage : |
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8 centigrade for one week. Store at -20 to -80 centigrade for twelve months from the date of receipt. |
| Storage Buffer : |
Supplied as solution form in PBS, pH 7.5 or lyophilized from PBS, pH 7.5. |
| Reconstitution : |
Reconstitute in sterile water for a stock solution. |
| Shipping : |
They are shipped out with dry ice/blue ice unless customers require otherwise. |
