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Goat Anti-CLN2 / TPP1 Antibody
Peptide-affinity purified goat antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, E |
|---|---|
| Primary Accession | O14773 |
| Other Accession | NP_000382, 1200, 12751 (mouse) |
| Reactivity | Human |
| Predicted | Dog |
| Host | Goat |
| Clonality | Polyclonal |
| Concentration | 100ug/200ul |
| Isotype | IgG |
| Calculated MW | 61248 Da |
| Gene ID | 1200 |
|---|---|
| Other Names | Tripeptidyl-peptidase 1, TPP-1, 3.4.14.9, 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. |
| Name | TPP1 |
|---|---|
| Synonyms | CLN2 |
| Function | Lysosomal serine protease with tripeptidyl-peptidase I activity (PubMed:11054422, PubMed:19038966, PubMed:19038967). May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases (PubMed:11054422, PubMed:19038966, PubMed:19038967). Requires substrates with an unsubstituted N-terminus (PubMed:19038966). |
| 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 |
Thousands of laboratories across the world have published research that depended on the performance of antibodies from Abcepta to advance their research. Check out links to articles that cite our products in major peer-reviewed journals, organized by research category.
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
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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