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Goat Anti-CLN2 / TPP1 Antibody

Peptide-affinity purified goat antibody

     
  • WB - Goat Anti-CLN2 / TPP1 Antibody AF1253a
    AF1253a (0.3 µg/ml) staining of Human Placenta lysate (35 µg protein in RIPA buffer) with (B) and without (A) blocking with the immunising peptide. Primary incubation was 1 hour. Detected by chemiluminescence.
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, E
Primary Accession O14773
Other Accession NP_000382, 1200, 12751 (mouse)
Reactivity Human
Predicted Dog, Cow
Host Goat
Clonality Polyclonal
Concentration 100ug/200ul
Isotype IgG
Calculated MW 61248 Da
Additional Information
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
StorageMaintain 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.
PrecautionsGoat Anti-CLN2 / TPP1 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
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
Research Areas
Citations (0)
citation

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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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$ 341.00
Cat# AF1253a
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