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.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
50 μg | ¥ 3,820 | 现货 | ||
100 μg | ¥ 6,530 | 5日内发货 | ||
200 μg | ¥ 11,130 | 5日内发货 | ||
500 μg | ¥ 22,510 | 5日内发货 |
生物活性 | Measured by the cleavage of AlaAlaPheAMC. The specific activity is > 850 pmoles/min/μg. |
产品描述 | 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. |
种属 | Human |
表达系统 | Baculovirus-Insect Cells |
标签 | His |
蛋白编号 | AAH14863.1 |
别名 | tripeptidyl peptidase I, CLN2, TPP-1, SCAR7, GIG1, LPIC |
蛋白构建 | 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. |
蛋白纯度 |
> 95 % as determined by SDS-PAGE
|
分子量 | Approxiamtely 60.7 kDa |
内毒素 | < 1.0 EU per μg of the protein as determined by the LAL method. |
缓冲液 | Supplied as sterile 20mM Tris, 500mM NaCl, pH 7.4, 10% glyPlease contact us for any concerns or special requirements. Please refer to the specific buffer information in the hard copy of CoA. |
复溶方法 | A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information. |
存储 |
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles. |
运输方式 |
Solution. It is shipped out with blue ice. |
研究背景 | 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. |
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TPP1 Protein, Human, Recombinant (His) tripeptidyl peptidase I CLN2 CLN-2 TPP1 SCAR 7 TPP-1 CLN 2 GIG 1 GIG-1 SCAR7 SCAR-7 GIG1 LPIC TPP 1 recombinant recombinant-proteins proteins protein