背景：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.
仕様
サイズ：50ug
種由来：Human
発現宿主：Baculovirus-Insect Cells
純度：＞95 ％ as determined by reducing SDS-PAGE.
エンドトキシン濃度：＜1.0 EU per μg of the protein as determined by the LAL method.
アクセッションNo.：AAH14863.1
アミノ酸配列：Met 1-Pro 563
遺伝子構造：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.
保存条件：Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at ＜ -20℃ for 3 months.
