Recombinant fragment, corresponding to amino acids 195-305 of Human TPP1
Abcam is committed to meeting high standards of ethical manufacturing and has decided to discontinue this product by June 2019 as it has been generated by the ascites method. We are sorry for any inconvenience this may cause. We would recommend antibody ab195234 as a replacement.
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 61 kDa.
Use a concentration of 3 µg/ml.
Use 2µg for 106 cells. ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
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.
Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.
Involvement in disease
Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2) [MIM:204500]. A form of neuronal ceroid lipofuscinosis. 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. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles.
Belongs to the peptidase S53 family.
Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.
Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease) antibody
Ceroid lipofuscinosis neuronal 2 late infantile antibody
CLN 2 antibody
GIG 1 antibody
Growth inhibiting protein 1 antibody
Lysosomal pepstatin insensitive protease antibody
Lysosomal pepstatin-insensitive protease antibody
TPP 1 antibody
TPP I antibody
Tripeptidyl aminopeptidase antibody
Tripeptidyl peptidase I antibody
Tripeptidyl-peptidase 1 antibody
Tripeptidyl-peptidase I antibody
IHC-P - TPP1 antibody (ab54685)
TPP1 antibody (ab54685) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human salivary gland.
Western blot - Anti-TPP1 antibody (ab54685)
Predicted band size : 61 kDa
Flow Cytometry - Anti-TPP1 antibody (ab54685)
Overlay histogram showing JEG3 cells stained with ab54685 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab54685, 2µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG1 [ICIGG1] (ab91353, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed.