|Application ||WB, E|
|Other Accession||NP_000382, 1200, 12751 (mouse)|
|Calculated MW||61248 Da|
|Other Names||Tripeptidyl-peptidase 1, TPP-1, 126.96.36.199, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, LPIC, Tripeptidyl aminopeptidase, Tripeptidyl-peptidase I, TPP-I, TPP1, CLN2|
|Format||0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||Goat Anti-CLN2 / TPP1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus (By similarity).|
|Cellular Location||Lysosome. Melanosome. Note=Identified by mass spectrometry in melanosome fractions from stage I to stage IV|
|Tissue Location||Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes 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. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.
Gene therapy for late infantile neuronal ceroid lipofuscinosis: neurosurgical considerations. Souweidane MM, et al. J Neurosurg Pediatr, 2010 Aug. PMID 20672930.
POT1-TPP1 enhances telomerase processivity by slowing primer dissociation and aiding translocation. Latrick CM, et al. EMBO J, 2010 Mar 3. PMID 20094033.
Late infantile neuronal ceroid lipofuscinosis: a new mutation in Arabs. Goldberg-Stern H, et al. Pediatr Neurol, 2009 Oct. PMID 19748052.
Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a Bid-dependent manner. Autefage H, et al. J Biol Chem, 2009 Apr 24. PMID 19246452.
Mutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis. Kousi M, et al. Brain, 2009 Mar. PMID 19201763.
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