Anti-TPP1 Antibody

This polyclonal antibody from Antibodies.com targets tripeptidyl peptidase 1 (TPP1), a lysosomal serine protease encoded by the TPP1/CLN2 locus on chromosome 11p15. Within the lysosome, TPP1 removes tripeptides from the N-terminus of substrate polypeptides and works alongside CLN5 and PPT1 to drive lysosomal protein turnover. It is the enzyme whose loss-of-function underlies neuronal ceroid lipofuscinosis type 2 (CLN2 disease, classic late-infantile Batten disease), making TPP1 a frequent target in pediatric neurodegeneration research, enzyme replacement therapy studies, and gene therapy trials using cerliponase alfa.

Raised in rabbit and supplied unconjugated at 1 mg/ml in PBS with 0.1% sodium azide and 50% glycerol, the reagent is shipped as a 100 µl vial. It has been validated for Western blot and immunohistochemistry, with reactivity confirmed against human, mouse, and rat samples, supporting cross-species work in tissue lysates, cultured fibroblasts, and iPSC-derived neuronal models.

Common uses include profiling endogenous TPP1 expression in patient and control fibroblasts, characterizing residual lysosomal protease activity in CLN2 mutation carriers, mapping TPP1 distribution in brain and retinal sections, and tracking biomarker responses in animal models of Batten disease. Sodium azide is included as a preservative, so handle with standard laboratory precautions.