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.
功能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.
组织特异性Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.
疾病相关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.
序列相似性Belongs to the peptidase S53 family.
翻译后修饰Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.
细胞定位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
Anti-TPP1 antibody 图像
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.
Anti-TPP1 antibody (ab54685)参考文献
This product has been referenced in:
Baradaran-Heravi A et al. Novel small molecules potentiate premature termination codon readthrough by aminoglycosides. Nucleic Acids Res44:6583-98 (2016).
Read more (PubMed: 27407112) »
Lojewski X et al. Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway. Hum Mol Genet23:2005-22 (2014).
Read more (PubMed: 24271013) »